Neurology Flashcards
Anton syndrome
Anton syndrome is due to bilateral parieto occipital infarcts causing cortical blindness with accompanying denial and confabulation.
Leber’s hereditary optic atrophy
Leber’s optic atrophy is a maternally transmitted mitochondrial disorder with identified mutations at positions 11778, 3460, 15257, 14484. Presentation is in young men with rapid permanent central vision loss.
Uhthoff’s phenomenon
It is associated with demyelination and optic neuritis. Temporary decrease in visual acuity with increased body temperature after a hot shower bath or exercise. Symptoms increase with rise in body temperature.
Patient can get into a hot bath but cannot get out.
Pulfrich phenomenon
It is seen in demyelination and optic neuritis. There is misperception of the trajectory of moving objects. Fading of vision might be there.
Cauda Equina Syndrome
Cauda Equina Syndrome - LMN
caused by the compression or injury to the nerve roots in the thecal sac of the lumbar spine and perineum (saddle anesthesia).
This syndrome presents as:
- low back pain
- motor and sensory deficits in the lower extremities (Flaccid)
- urinary retention
- fecal incontinence
Cauda equina syndrome can also present due to an intradural extramedullary abscess. If a spinal abscess with epidural collection is suspected, then a lumbar puncture is contraindicated.
Tabes Dorsalis
tabes means wasting away
Tabes Dorsalis - occurs 10-35 yrs after primary syphillis
- dorsal sensory columns degenerates
- Rhomberg becomes positive
- Argyl Robertson pupil present
- Bilateral forehead wrinkling + ptosis
- General hypotonia, Absent reflexes and absent or extensor plantars
- Deep achilles tendon pain absent
- Charcot joints
Other Features rare now:
- Wide based high stepping gait of sensory ataxia
- Zones of cutaneous analgesia
- Ligament laxity allowing extremes of leg movements
- Foot ulcers
- Lightening pains along limbs
- Pel’s crisis: Ocular crises in tabes dorsalis: paroxysmal sudden pains affecting the eyes + tearing + photophobia
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D/d - Freidrich ataxia, MS
General paralysis of insane
General paralysis of insane
- feature of neurosyphillis
- Early dementia progresses to euphoria
- delusions of grandeur
- Epileptic fits
- Hand, Lip, Tongue tremors (trombone tremor)
- tongue darts in and out involuntarily
- Spastic paraparesis
Meningo-vascular syphillis
Meningo-vascular Syphillis
- isolated cranial nerve palsies (3, 6 common)
- Cerebral or spinal stroke
- Meningism
- Epilepsy
Other Rare features:
- syphillitic amyotrophy - resembles MND
- Meningomyelitis
- Pachymeningitis
- Acute transverse myelitis
- Erb’s spastic paraplegia
- Endarteritis with aortitis, aneurysm
Circle of willis
Circle of willis
MCA splits into 3 - ACA, PComA, Main MCA
Branches- Ophthalmic + ant choroidal artery
Anterior Cerebral artery joins by Ant communicating Art
Posterior communicating artery joints PCA
Posterior Cerebral artery
2 pca joints to form basilar
basilar artery with pontine perforating branches
branch - AICA, PICA
2 vertebral arteries joins other end into basilar
Subclavian Steal syndrome
Subclavian steal syndrome
- stenosis at origin of subclavian artery on R/L side
- Blood flow diverts to vertebral artery, specially during exercise due to this stenosis and BP will be low on affected side and features of vertebro-basilar insufficiency will be present as:
- Vertigo, Syncope,
- Auditory hallucinations
- Diplopia
- Bilateral blurring of vision or transient bilateral blindness
- Affected arm easily tires during exercise
- fainting attacks while drying out laundry or taking out stuff from top shelf
- Bruit over subclavian artery
Parasympathetic Ganglions of Head - SPOC - 7793
SPOC-7793: Parasympathetic ganglions of head, numbers are for cranial nerve involved.
1. Submandibular Ganglion:7
Preganglionic - 7th facial nerve (chordaTympani branch)
PostGanglionic - directly to sublingual and submandibular glands
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2. Pterygopalatine Ganglion: 7
Preganglionic - 7th nerve, Greater petrosal branch
Postganglionic - V2 branch of Trigeminal,
To - Nasopharynx, Lacrimal, Palate
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3. Otic Ganglion: 9
Preganglionic: 9th, Lesser petrosal N.
Postganglionic: Auriculotemporal N. to parotid gland
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4. Ciliary Ganglion: 3
Preganglionic: 3rd, Oculomotor nerve
Postganglionic: Short ciliary nerves to sphincter pupillae and ciliary muscles
Superior Orbital fissure contents
contents of Superior Orbital Fissure:
it is in sphenoid bone at upper orbit
- Superior Ophthalmic vein
- Inferior Ophthalmic vein
- Oculomotor nerve, 3
- Trochlear nerve, 4
- Abducens nerve, 6
- Branches of Opthalmic division V1:
1.Lacrimal nerve
2. Frontal nerve
3. Nasociliary nerve
Inferior Orbital Fissure contents
Inferior Orbital fissure is in sphenoid and maxilla,
what passes through it:
- Inferior ophthalmic Vein
- Infra Orbital artery
- Infra Orbital vein
- Zygomatic branch of V2
- Infra orbital branch of V2
- Orbital branches of Pterygopalatine ganglion (remember SPOC 7793)
Jugular foramen contents
Jugular foramen is in occipital & temporal bones & contents are:
- 9, 10, 11th cranial nerves
- Posterior meningeal artery
- Ascending pharyngeal artery
- Inferior petrosal sinus
- Sigmoid sinus
- Internal jugular vein
Branches of External Carotid Artery -
Mnemonic:
Some Anatomists Like Freaking Out Poor Medical Students
Branches of External Carotid Artery - Mnemonic: Some Anatomists Like Freaking Out Poor Medical Students
1. Superior Thyroid Artery
2. Ascending pharyngeal artery
3. Lingual artery
4. Facial artery
5. Occipital artery
6. Posterior auricular artery
7. Maxillary artery - to ear, nose, teeth, TM, Palate - middle meningeal artery
8. Superficial temporal artery
Pterygoid venous plexus communications
Important communications of pterygoid venous plexus are:
- Anterior Facial vein
- Inferior ophthalmic vein
- Maxillary vein to Retromandibular vein to external subclavian vein to Jugular vein
- cavernous sinus through emissary veins
branches of ICA - Internal carotid artery
A VIPS COMMA
A VIPS COMMA - branches of ICA
A- Anterior choroidal artery
V- Vidian artery
I - Inferolateral trunk
P- Posterior communicating artery
S- Superior Hypophyseal artery
C- Carotid tympanic artery
O- Ophthalmic artery
M- Meningo-Hypophyseal trunk
M- MCA
A- Anterior Cerebral artery
Dystrophia myotonica - clinical features - ABCDEFGH
1 form of muscular dystrophy
Mnemonics as ABCDEFGHI
A - Auto Dominant, by 20-30 yrs
B - Balding (frontal)
C - Cardiomyopathy,
C - Cataracts,
C - CTG repeats upto 1000 in DNA
D - Diabetes, Distal myopathy
E - Expressionless face (myotonic)
F - Facial muscle dystrophy
G - Gonadal (Testicular) atrophy
H - Heart block,
H - Hypo-Gamma-globulinaemia
I - Infections, recurrent
sagging sad face due to poor muscle tone
Trigeminal nerve branches
Trigeminal nerve, 5th cranial nerve, pontine origin, Sensory, Has 3 main divisions:
V1, Opthalmic Division: branches into
- SupraOrbital nerve
- SupraTrochlear nerve
- Infratrochlear nerve
- External nasal nerve
- Lacrimal nerve
V2, Maxillary Division, branches into
- Infraorbital nerve
- Zygomaticofacial nerve
- Zygomatico-temporal nerve
V3, Mandibular Division, branches into
- Mental nerve
- buccal nerve
- Auriculo-temporal nerve
Abducens nerve
Abducens, 6th cranial, pontine origin, longest course, supplies Lateral Rectus
Palsy causes weakness of lateral eye movements - with diplopia on looking laterally (side of palsy)- outer image disappears on covering weak eye
Causes of palsy:
- Stroke, Trauma, Raised ICT, DM, MS
- Lyme disease, Syphillis, TB, Meningitis
- Vasculitis, Sarcoidosis, SLE
- Orbital tumor, cellulitis,
If abducens palsy is noted - check Limb power and Deep tendon reflexes to rule out cortico-spinal involvement
Cervical Spondylitis
Cervical spondylitis:
- Cord myelopathy due to disc herniation, osteophytes, bony spurs
- Starts as neck, hand pain, paraesthesia referred to scapula & progresses to:
- Gait problems
- Urinary issues - retention/incontinence
- gradual spastic paraparesis,
- Cough, straining causes sudden leg weakness
- Loss of Bicep DTR’s
- Wasting of Hand muscles, C5C6 common
Leg Deep Tendon Reflexes and
Babinski/Chaddock/Oppenheim/Gordon signs
DTR’s in Leg
Knee DTR - L3L4 - tap quadriceps
Ankle DTR - L5S1 - tap achilles
Plantar reflex - scratch along lateral sole to(down to up), also called babinski reflex - upgoing is UMN sign, absent in tabes dorsalis
Chaddock sign - stroke foot laterally below malleolar skin back to front and look for babinski response - if up going - UMN lesion suspected
Oppenheim Sign - stroke with fingers along anterior tibial shin downwards, babinski like upgoing response indicates UMN lesion
Gordon Sign: - firm squeezing of calf muscle firmly shows babinski like upgoing response- suspect UMN lesion
Foot movements-Muscles-Nerves
Foot movements-Muscles-Nerves
Plantar flexion - by Gastrocnemius, soleus - S1 Sciatic
Foot Dorsi flexion - Tibialis anterior, Long foot extensors, L4L5 (common peroneal-sciatic)
Foot Inversion - Tibilais anterior, Tibialis posterior - L4, Tibial nerve
Foot Eversion - Peronei muscles, S1, common peroneal-sciatic
Great Toe Extension = Extensor hallucis longus - L5 root
Lumbo-Sacral PLexus
Lumbo-Sacral Plexus of nerves
Major nerves - FOSIPS (6)
- Femoral nerve L2L3L4
- Obturator nerve L2L3L4
- Superior Gluteal Nerve - L4L5S1
- Inferior Gluteal nerve L5S1S2
- Pudendal nerve S2S3S4
- Sciatic Nerve L4L5S1S2
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Minor branches of this plexus 11
- Iliohypogastric nerve
- Ilioinguinal nerve
- Genitofemoral nerve
- Lateral cutaneous nerve of thigh
- Nerve to quadratus femoris
- Nerve to inferior gemellus
- Nerve to obturator internus
- Nerve to superior gemellus
- Nerve to piriformis
- Nerve to Levator ani
- Nerve to external anal sphincter
Histamine Response Test for nerve lesion
Histamine Response Test is for determination whether a nerve lesion is pre ganglionic or post ganglionic-
Procedure - a drop of 1% Histamine is applied to affected dermatome and skin is pricked through it.
Normal Response = Triple flare within 10 min
Post Ganglionic Lesion = no response
Pre Ganglionic Lesion = persistent flare response even at 3 weeks
Tonic Clonic meaning
Tonic Clonic Seizures
A tonic seizure causes a sudden stiffness or tension in the muscles of the arms, legs or trunk. The stiffness lasts about 20 seconds and is most likely to happen during sleep. Tonic seizures that occur while the person is standing may cause them to fall.
Clonus” (KLOH-nus) means fast stiffening and relaxing of a muscle that happens repeatedly. In other words, it is repeated jerking. The movements cannot be stopped by restraining or repositioning the arms or legs. Clonic (KLON-ik) seizures are rare and most commonly occur in babies.
Tonic-clonic seizures involve both tonic (stiffening) and clonic (twitching or jerking) phases of muscle activity. Tonic-clonic seizures may start with a simple partial seizure or aura. The person may experience changes in sensation, mood or emotion leading up to the tonic-clonic seizure.
Rule of 2 for Lumbar Plexus
Rule of 2 of Lumbar plexus
2 roots supplies 1 action and next 2 other opposite action.
At Hip Joint:
Flexion by L2L3 - Iliopsoas muscle
Extension by L4L5 - Glutes & Hamstrings
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At Knee Joint:
Extension: by L3L4 - Quadriceps
Flexion: by L5S1 - Hamstrings
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At Ankle Joint:
Flexion by L4L5 - Dorsiflexion, Posteriaris tibialis extensor
Extension: S1S2 - Plantar flexion, Calf muscles
Ulnar Nerve
Ulnar Nerve, Medial cord, C8T1, Sensory to 2 and motor to 6
Muscles supplied:
1. FCU - flex wrist & extend against resistance to feel FCU tendon
2. FDP - ULNAR 1/2, flex distal phalanx of little finger against resistance
3. Hypothenars-abduct little finger against resistance
4. Interossei - Hold paper between little and ring finger tightly, dont let pull
5. Medial 2 lumbricals
6. Adductor pollicis - grasp paper between index & thumb, weak AP makes thumb to flex to hold paper-known as Froment or Journal Sign
Wardenberg sign - Involuntary abduction of little finger due to weakness or paralysis of the adducting palmar interosseous muscle and unopposed action of the radial innervated extensor muscles (digiti minimi, digitorum communis ).
Treatment of isolated Wartenberg’s sign consists of re-routing the extensor digiti minimi. Among the other techniques, Belmahi’s “tie lasso” is preferred when flexible claw hand is associated with Wartenberg’s sign
Wardenberg Sign
Wardenberg sign - Involuntary abduction of little finger due to weakness or paralysis of the adducting palmar interosseous muscle and unopposed action of the radial innervated extensor muscles (digiti minimi, digitorum communis ). Lesion is proximal to wrist.
Treatment of isolated Wartenberg’s sign consists of re-routing the extensor digiti minimi. Among the other techniques, Belmahi’s “tie lasso” is preferred when flexible claw hand is associated with Wartenberg’s sign
High Ulnar Lesion
Ulnar paradox
Less obvious claw hand as FDP paralysed and doesnot flex DIP so now claw obvious. Lesion is at elbow or at cubital tunnel or ulnar groove
Ulnar claw Hand (Low Lesion)
Distal digits of index, middle fingers flexes but of ring + little fingers do not (ulnar side) -when lesion is beyond wrist. Muscles innervated by the ulnar nerve:
- Abductor digiti minimi.
- Flexor digitorum profundus.
- Flexor digiti minimi.
- Opponens digiti minimi.
- Ring finger lumbricals.
- Small finger lumbricals.
- Dorsal & palmar interosseous muscles.
- Adductor pollicis.
Primary Site of lesions:
1. Ulnar tunnel between pisiform & hook of hamate Or
2. at Guyon’s canal at wrist
3. Medial epicondyle
4. Brachial plexus injury
Median Nerve
Supplies LOAF muscles in Hand
= Lumbricals, 1 & 2
= Opponens pollicis
= Abductor pollicis brevis
= Flexor pollicis brevis
Median Nerve, Lateral +Median cord
C5C6C7C8T1 roots, Feel between tendon of FCR & palmaris longus@wrist
Sensory to Radial 3&1/2 fingers
Motor to 9 muscles
1. Pronator teres - palm up-to palm down against resist
2. FPL to thumb-distal phalanx flexes
3. FDP - distal phalanx flexes of 2+3no
4. APB - thumb abduction against resist
5. Palmaris longus
6. Flexor digitorum superficialis and
7. Flexor carpi radialis
Clinical relations: Carpal tunnel syndrome, hand of Benediction, simian/ape hand deformity, pronator syndrome
Carpal Tunnel Median Neuropathy
Tingling numbness beyond wrist or base of thumb
Thenar wasting
Numbness on lateral 3 & 1/2 fingers on all palm side and only distally on dorsum of fingers.
Tinel’s Sign: Tap on wrist for symptoms
Phalen’s Sign: wrist flexion induces symptoms
Hyperalgesia in median nerve territory
Katz Hand Diagram: pt draws area of numbness
Weakness of abductor pollicis brevis (thumb)
Lesion above Elbow: Hand of benediction, (thumb + index extended) + lateral forearm wasting
Anterior Interosseus Branch lesion: isolated weak pincer grip
Rx:
Splinting at wrist, specially at night
Steroid injections into wrist
Yoga techniques
NCV study & decompression (open/ Endoscopic)
Radial Nerve Testing
Radial Nerve - extensor nerve
Sensory to small patch skin on dorsum between thumb & index & post arm
Motor to all extensor muslces of ARM+Forearm+Hand
- Triceps testing @ elbow extension C7C8
- Wrist extension against resistance
- Finger extensions
- Elbow flexion with midpronation-Brachioradialis
- Supination against resistance
Radial palsy will cause wrist drop
Common sites of injury:
= at elbow or below elbow: #’s, dislocation, ganglions
= Shaft of humerus fracture - in groove
= Axilla: crutches, Saturday Night palsy due to overnight stupor sleep over arm of chair pressing radial nerve
Low Lesion: Tricep Jerk preserved
High Lesion: Tricep jerk Lost
C7 root lesion: Tricep DTR lost + wrist drop + weak shoulder adduction + weak elbow extension
Pre-trunk branches of Brachial plexus
Pre-trunk branches of brachial plexus
1. Dorsal scapular nerve - to rhomboids (C5) - test: ask to put both hands on hips - push elbow forward against resistance - feel rhomboid on back
2. Long thoracic nerve - to Serratus anterior muscle - ask to put both hands on a wall and push it away - look for winging of scapula in weak muscle
3. Phrenic nerve - C5C6C7, innervates diaphragm - do sniff test
Sniff test for Phrenic nerve function
The fluoroscopic sniff test, also known as diaphragm fluoroscopy, is a quick and easy real time fluoroscopic assessment of diaphragmatic motor function (excursion). It is used most often to confirm absence of muscular contraction of the diaphragm during inspiration in patients with phrenic nerve palsy or breathing difficulties following stroke. Chest radiograph demonstrating a newly elevated hemidiaphragm often precedes a sniff test.
Technique = ask the patient to practice sniffing before the study with the patient either standing (preferred) or supine, perform frontal fluoroscopy of the diaphragm at rest, breathing quietly through an open mouth ask the patient to take a few quick short breaths in with a closed mouth (‘sniffs’) causing rapid inspiration occasionally, repeating (3) in the lateral projection is required to evaluate the posterior hemidiaphragms
Findings = In normal diaphragmatic motion:
the diaphragm contracts during inspiration: moves downwards
the diaphragm relaxes during expiration: moves upwards both hemidiaphragms move together in healthy patients 1-2.5 cm of excursion is normal in quiet breathing = 3.6-9.2 cm of excursion is normal in deep breathing 2 up to 9 cm can be seen in young or athletic individuals in deep inspiration 2 excursion in women is slightly less than men 2
In abnormal diaphragmatic motion:
the affected hemidiaphragm does not move downwards during inspiration
paradoxical motion can occur
Interpretation = Absence of diaphragmatic movement confirms phrenic nerve palsy in the appropriate clinical setting. A mass anywhere along the course of the phrenic nerve requires further workup, usually with neck and chest CT. A hilar mass due to lung cancer is the most common finding on CT and a classic exam case. Normal diaphragmatic excursion can also be impaired in patients with: previous diaphragmatic trauma or surgery neuromuscular disorders
previous stroke
Trunk Level Branches of brachial plexus
Trunk Level branches of brachial plexus
1. Supra scapular nerve to supra-spinatus, infra spinatus - test by shoulder abduction and rotation (rotator cuff function)
2. Nerve to subclavius - C5C6 -
The subclavius muscle is a short, triangular muscle of the thoracic wall that lies underneath the clavicle. It originates from the first rib and courses laterally to insert on the undersurface of the middle third of the clavicle. The main function of the subclavius is to stabilize the clavicle during movements of the shoulder girdle. In addition, it is significant in preventing injuries to the adjacent neurovascular structures due to fractures of the clavicle.
Costo-clavicular syndrome
Costo-clavicular syndrome
The vessels and nerves running behind the subclavius muscle can sometimes become entrapped between the clavicle and the first rib, inside the costoclavicular space. This is referred to as costoclavicular syndrome and marks one of the three types of thoracic outlet syndromes (TOS).
This syndrome can be caused by fractures and thoracic deformities, amongst others. Typical symptoms include irritations of the brachial plexus and circulatory disorders of the arm
Brachial Plexus
Brachial means pertaining to upper limb
Brachial plexus - 3-6-3=3-5-5
Trunks 3, Divisions 2x3=6, Cord 3
Trunks:
- Upper, C5C6
- Middle, C7
- Lower, C8T1
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Divisions - Anterior and Posterior, 2x3=6
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Cords: - 3
Lateral - joining of Anterior division of Upper trunk & Anterior division of lower trunk - Has 3 branches
Medial Cord - posterior division of Lower trunk - Has 5 branches
Posterior Cord - all posterior division of all trunks input to this one - Has 5 branches
Radial Nerve Palsy: Wrist Drop
Radial Nerve Palsy - Posterior Cord, Extensor to whole arm - 2 sites of injury
1. Axillary Radial injury -
- Triceps extension & Reflex both lost
- Brachioradialis extension lost
- Supination lost
- Wrist Extension + MCP extension lost
2. Mid arm injury against Humerus - Saturday Night Palsy-recovers in weeks. Brachioradialis, Supinator, Wrist and MCP extension lost but Tricep preserved.
# First dorsal interossei skin numbness
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# If wrist is passively extended, Power grip improves and person can extend IP joints but cannot extend (still) MCP joints.
# Finger adduction & abduction normal if hand placed flat on a table with radial action eliminated (as lumbricals & Interossei normal)
Pseudobulbar Palsy: Features
Pseudobulbar UMN Features in brainstem distribution
- salivating at angle of mouth
- Unable to protrude tongue, lies in mouth, small and spastic
- Donald duck speech, High pitch, slurred, monotonous
- Dysphagia with nasal regurgitation @swallowing attempts
- Palatal Movements absent
- Exaggerated Jaw jerk reflex
- Bilaterally Generalised spasticity,
- Extensor Plantars
- Emotionally labile
- Bilateral internal capsule stroke is the most common cause
Pseudo-Bulbar Palsy: Causes
Pseudo-Bulbar Palsy - resembles a bulbar palsy but due to UMN cause
- Bilateral IC strokes is most common
- Multiple sclerosis
- Motor neuron disease
- High Brainstem tumors
- Head injury
Lateral Medullary Syndrome(Wallenberg, PICA)
Posterior Inferior Cerebellar artery occlusions - Posterior circulation Stroke
Lateral Medullary-PICA syndrome
Face numb + oppositie body numb
Structures affected: ipsilateral
= Tractus solitarius - loss taste
= Sympathetic pathways - Horner’s
= Cerebellar connections - cerebellar signs - DANISH
= 5th nerve nucleus-same side face numb
= 9, 10th nerve - palatal paralysis, no gag reflex, no palatal movements
and:
= Spino-thalamic tract: contralateral loss of pain & temperature sensation
Hiccups
Vertigo, nystagmus, Nausea, Vomiting (vestibular nuclei affected)
Medial Medullary Syndrome (Dejerine)
Medial Medullary Syndrome: -
The medial medullary syndrome is a result of blockage of the anterior spinal artery or medial medullary branches of the vertebral artery. It affects the pyramid, medial leminiscus and hypoglossal nerve resulting in contralateral hemiparesis of the limbs, ipsilateral lingual paralysis, and contralateral impaired perception of joint position, vibration and discriminative touch.
Basal Pontine Infarction (Millard-Gubler)
Basal infarction of the pons = follows occlusion of pontine branches of the basilar artery. Corticospinal fibres are dispersed at this level and partial contralateral hemiplegia results. Abducent nerve damage paralyses the lateral rectus muscle. If the lesion extends dorsally the facial nerve may be affected.
Internuclear Ophthalmoplegia (INO)
Internuclear ophthalmoplegia arises when the medial longitudinal fasciculus is involved either in a vascular lesion or in a demyelinating disease such as multiple sclerosis. Ipsilateral 3rd nerve connection to contralateral 6th nerve nucleus is lost affecting conjugate horizontal eye movements.
Weber Syndrome - Basal Midbrain Lesion, POCS, PCA occlusion
Superior alternate Hemiplegia
thrombosis of central branches of the posterior cerebral artery (PCA), affecting = corticospinal and corticobulbar fibres in the basis pedunculi, with contralateral hemiplegia.
Destruction of the oculomotor nerve causes severe ipsilat ophthalmoplegia, only the lateral rectus and superior oblique muscles escaping.
= Ipsilateral 3rd nerve: Down, Out eye with dilated fixed pupil
= Contralateral Hemiplegia
Nothnagel’s syndrome,
(Midbrain Lesion)
Midbrain lesion
Ipsilateral 3rd nerve palsy
Cerebellar ataxia
Foville’s syndrome, Pons
Foville’s syndrome
Pontine Lesion = inferior medial pontine stroke syndrome that most commonly presents with contralateral hemiparesis and ipsilateral abducens and facial palsies.
Same as Millard-Gubler but with lateral conjugate gaze palsy
Jugular foramen syndrome, 9,10,11
Jugular foramen syndrome: - Palsies of 9, 10, 11 cranial nerves
- Loss of taste along posterior 1/3rd of tongue
- Reduced Gag & palatal movements
- Uvula deviated to other normal side
- Weak wasted sternocleidomastoid
- Weak shoulder shrug & looks flat
Look for brainstem signs as well such as:
- wasted fasciculated deviated tongue
- Ipsilateral horners syndrome
-Spastic limb paraparesis
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Causes:
= Neurofibroma of 9,10,12 nerves
= Meningioma
= Epidermoid tumors
= Glomus or carotid body tumors
= Metastasis in the area
= CP angle lesions with 8th nerve +
= Middle ear infection to post fossa
= Cholesteatoma
= Granulomatous meningitis
Syndrome of Vernett
Vernet syndrome refers to paralysis of the IX, X, and XI cranial nerves traversing the jugular foramen. A variety of lesions can involve the jugular foramen, such as tumors, vascular lesions, infections, and trauma
Syndrome of Collet-Sicard
Collet-Sicard syndrome (CSS) is a condition showing unilateral paralysis of lower cranial nerves (CN) IX, X, XI, XII. It was first described by Collet, in 1915, in a World War I soldier with a bullet injury in the mastoid region. Hypoglossal foramen is near external opening of jugular foramen and external lesions can involved 9+10+11+12 cranials.
Syndrome of Villaret
Unilateral lesion affecting nIX to nXII, Collet-Sicard syndrome, in combination with ipsilateral Horner syndrome is called Villaret syndrome. Lesions affects 9,10,11,12 and cervical sympathetic chain as well.
# 10+11 nerves only affected - Syndrome of Schmidt
# 10+11+12 nerves affected - Syndrome of Hughlings Jackson
Infantile Hemiplegia
Infantile Hemiplegia - some CNS insults in first yr of life/ birth/ Fetus leads to unilateral hemiplegia with some times Mental retardation.
- Encephalitis
- Toxic Encephalopathy
- Infections as Measles, Pertusis, Scarlet Fever
Features:
- Unilateral small limbs with weakness
- Epilepsy
- Chorioid or athetoid limb movements
- Ankle clonus, Jaw Clonus
- Limb spasticity
- Brisk reflexes on weak side as UMN
- Spastic Dysarthria
Horner’s Syndrome - Sympathetic chain
Horner’s Syndrome features:
= Ipsilateral meiosis - due to excess parasympathetic activity
= Enophthalmous (smallish eye)
= Anhidrosis (no forehead sweating)
= Partial Ptosis same side
——————————– causes are as:
Carotid Dissection
Vertebral Dissection
Brainstem strokes - wallenberg syn.
Multiple sclerosis
Syringomyelia
Neck surgery or trauma
Neck invasive pancoast tumor
Neck Lympnodes affecting sympathetic
Carotid artery aneurysm
Clinical triad of Carotid Dissection
Clinical Triad of Carotid Dissection
1. Partial Horner’s syndrome +
2. Pain on same side of face, neck, head
3. Later Cerebral or retinal ischemic signs
typical history of - hyperextension or rotation of neck as - painting the ceiling, coughing, vomiting, Sneezing, post anaesthesia administration.
Rx by anticoagulation if no intra cranial extension.
SACD - Subacute Combined Degeneration of the Cord
SACD features:
= Peripheral paraesthesias (burning soles). unsteady gait
= Absent ankle DTR + Extensor plantars
= Stocking & Glove sensory loss
= Rhomberg sign positive
= Dorsal column degenerates, vibration, Position, Touch lost
= Lhermitte sign
= Lemon yellow skin pallor-B12 deficient anemia
= Glossitis, Spleenomegaly, Ca stomach,
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SACD causes:
= Addisonian pernicious anemia, Autoimmune associations
= Total/Partial gastrectomy
= Stagnant loop syndrome
= Ileal resection or Crohn’s disease
= Strict vegan diet (Lacks B12)
= Fish Tapeworm
= Chronic Tropical sprue
= Congenital Intrinsic factor deficiency
Lhermitte phenomenon
Lhermitte phenomenon:
electrical sensation down the spine into the legs on flexing the neck
seen with
= Multiple sclerosis
= SACD
= Cervical Spondylosis
= Cervical cord tumors
Reverse Lhermitte sign in neck extension is strongly seen in Cervical spondylosis
Dysarthria - speech issue, broken
Dysarthria varieties:
1. Cerebellar dysarthria - slow staccato scanning speech - reading each syllable slowly, look for other cerebellar signs-DANISH
2. Spastic Dysarthria - tongue stuck in mouth - hot potato or Donald duck speech, High pitched, rigid parts
= Pseudobulbar palsy
= Parkinsons disease
= Myotonic dystrophy
= Huntington’s chorea
= General paralysis of insane
3. Flaccid dysarthria:
= Bulbar palsy
= Stroke involving 7,9,10, 12 nerves
4. Myopathic dysarthria: weak muscles
= Myasthenia gravis - worse with repeat
5. Structural dysarthria:
= Hypothyroidism, low pitch, guttaral,
= Amyloidosis, large tongue
= Multiple oral ulcers or painful thrush
= Parotitis or TM joint arthritis with pain
Facial nerve Palsy - clinical
Clinical features of Facial Palsy
- frontalis forehead wrinkling lost LMN
- Bell’s Phenomenon - protective as corneal exposure minimized - eye turns upward when closed
- Nasolabial angle flattening
- Unable to close eye completely
- angle of mouth flattened
- Anterior 2/3rd of tongue lost taste
- Hyperacusis due to stapedius palsy
Facial Nerve Palsy - Causes
Unilateral Facial palsy
= Bell’s palsy (idiopathic)
= CP angle tumors (5,6,7,8 all affected) Acoustic neuroma, meningioma
= Parotid Tumors
= Herpes zoster also affects 5, 8 nerves
= Pontine lesion - MS, stroke etc
= Middle ear- otitis, cholesteatoma
= Mononeuritis multiplex: due to DM, PAN, Churg-strauss, Wegner’s, Sarcoid, Carcinoma, SLE, RA, Amyloidosis, Leprosy
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Bilateral Facial Palsy:
= Lyme disease
= Bilateral bell’s palsy
= Guillain Barre syndrome
= Myasthenia gravis
= Congenital facial diplegia
= Motor neuron disease, rare
= Sarcoidosis
Tardive dyskinesia
Means late happening abnormal movement
Tardive dyskinesia - means late happening abnormal movements like lip smacks, chewing, pouting, grimacing (orofacial-dyskinesia) - there might be choreoathetosis of the limbs and trunk.
It started later upto an year after starting or stopping medications as Phenothiazines.
Neuroleptics causing dyskinesia:
= Phenothiazines (chlorpromazine)
= Butyrophenones (Haloperidol)
= Substituted Benzamide - metoclopramide
= Reserpine
= Tetrabenazine
Other abnormal movements due to Neuroleptics:
= Acute dyskinesias-soon after drug
= Akathisia - uncontrollable restlessness with inner feeling of unease
= Parkinsons syndrome - responds to anticholinergics rather than L-DOPA
Carpal Tunnel Syndrome - Causes
CTS - Causes of Median Neuropathy
- Amyloidosis of chronic diseases as
- Hypothyroidism, Myxoedema
- Rheumatoid arthritis
- Pregnancy, OC pills
- Acromegaly
- Osteoarthritis of the carpus
- Primary Amyloidosis, thick nerves
- Trophaceous Gout
- Tuberculous tenosynovitis
Internuclear Ophthalmoplegia INO
It is due to Lesion of medial longitudinal fasciculus which connects 3rd nerve nucleus to opposite 6th nerve nucleus, usually due to demyelination of MS.
Clinical - adduction failure on affected side (RE adduction failure with Right MLF lesion) - and nystagmus and diplopia. When other eye is covered - adduction is normal.
Causes:
- MS
- Diabetic vascular lesions
- Werrnicke’s encephalopathy
- SOL
Wernicke’s encephalopathy
Acute Thiamine (B1) deficiency causes it, usually in alcoholic but also possible in Hyperemesis gravidarum, Malnutritions - triad of ocular dysfunction, ataxia, and altered mental status.
- Ocular palsies ie ophthalmoplegia
- Nystagmus
- Loss of pupillary reflexes
- Ataxia
- peripheral neuropathy
- Korsakoff psychosis, irreversible
Diagnostic Triad of Wernicke Encephalopathy
Encephalopathy
= Disorientation
= Inattentiveness
= Apathy
= Decreased level of consciousness
= Memory Impairment
Ataxia
= Wide-based gait
= Inability to stand
Ophthalmic abnormalities
= Gaze-evoked nystagmus
= Impaired abduction
= Conjugate gaze palsy
= Internuclear ophthalmoplegia
= Impaired horizontal vestibulo-ocular reflex
= Optic disc edema
= Retinal hemorrhage
Nystagmus Classification
Nystagmus Types:
- Physiological nystagmus
- Ocular nystagmus
- Vestibular nystagmus
- Cerebellar nystagmus
- Ataxic nystagmus
Vestibular Nystagmus
Vestibular Nystagmus - can be
1. Peripheral: Lesion in labyrinth or vestibular nerve =
= Fast component to opposite of lesion
= Fatiguable
= Unsteadyness on same side
= Open eye unsteadyness
= Vertigo, Deafness
2. Central - Lesion in Vestibular nucleus and its connections (Medullary)
= Spontaneous but can with head spin
= not fatiguable
= Has vertical component usualy
= Downbeat can be
Peripheral Vestibular Nystagmus:Cause
Causes of Peripheral vestibular nystagmus are as:
= Labyrinthitis
= Menier’s syndrome
= Acoustic neuroma
= Vestibular neuronitis with or without deafness
= Middle ear degenerative disease
= Hypertension
= Head injury
Central Vestibular Nystagmus-Causes
Causes of central Vestibular nystagmus are as :
= MS, CVA
= Encephalitis
= Tumors
= Syringobulbia
= Alcoholism
= Anticonvulsants
—————————————
Arnold-Chairy malformations - has downbeat nystagmus while looking straight ahead
—————————–
Formaen Magnum lesions as tumors, syringomyelia, cerebellar degeneration - downbeat nystagmus on lateral gaze
Holmes-Adie-Moor Syndrome
Holmes-Adie-Moore Syndrome:
= Very Slowly reactive (tonic)pupil to Light - remains dilated in light - Doesnt constrict fast - in dark also do not dilate fast
= constrict slowly to accomodation/ convergence and might become smaller than other normal one
= Absent DTR’s
acute development might cause - blurring and photophobia
= no eye palsy, no ptosis, no diplopia (no features of 3rd nerve palsy)
= Hyperreactive to Pilocarpine
= Normal reaction to mydriatics
Syringomyelia - upper cord
Syringomyelia - upper cord syrinx
- Bilateral loss to Pain and heat
- Intact to cold but not warm(early)
- UL: absent DTR’s, wasted ulnar forearm, painless burns & cuts, weak and wasted small Hand muscles
- LL: exaggerated DTRS, extensor plantars (Spastic paraparesis)
- Kypho-scoliosis
- Horner’s syndrome may be present
expanding syrinx destroys spino-thalamic tract neurons first as they are more medial than other hence pain and temp lost (warm lost earlier than cold)
Syringobulbia - brainstem
Syringobulbia - when syrinx extends up into brainstem or starts there
= Nystagmus
= Ataxia
= Lower Cranial nerve palsies
= Onion skin facial sensory loss with peripheral lost first
= Bulbar Palsy: Wasted tongue with fasciculations, weak SCM+Trapezius, numb face, dysphasia, dysarthria, Palatal palsy, gag weak, aspiration risk
= La main succulente: cyanosed puffy cold hands with stumpy fingers
= Lost or excessive sweating areas
= Charcot Jts: Elbow, Shoulder
Syringomyelia Associations
Associations in Syringomyelia, may be
= Fused cervical vertebrae (Klippel-Feil syndrome) with short neck
= Kypho-scoliosis
= Asymmetrical thorax
= Sternal depression or Prominence
= Cervical ribs
Myasthenia gravis - Clinical
MG - clinical features
- snarling weak face
- ptosis, B/l or unilateral
- Diplopia with squint
- Fatiguable limb weakness
- SOB dangerous, monitor FVC
- Proximal myopathy features
- Jaw supporting sign
- Anti AcH reception antibodies 90%
- Do Tensilon test (Edrophonium), muscle improves after it
MG associations
Associated conditions with Myasthenia
- Thyrotoxicosis, 5%
- Hypothyroidism
- Rheumatoid Arthritis
- Sarcoidosis
- Diabete mellitus
- Polymyositis, dermatomyositis
- SLE
- Pernicious anemia
- Sjogren’s syndrome
- Pemphigus
Myasthenic Crisis
Myesthenic crisis, Chrolinergic crisis
- Nausea, vomiting
- Salivation, sweating
- Lacrimation
- Miosis
- Pallor
- Breathlessness due to weakness (FVC)
- Collapses, confusion
Can be triggers by - emotional stress, infections, stress, drugs as Aminoglycosides, Colistin, Polymyxin, Curare, Quinine, Quinidine etc
Lambert Eaton Myasthenic syndrome
Eaton Lambert:
- Malignancy associated MG like (oat cell Ca of bronchus)
- Fatiguability improves with exercise
- Absent Tricep reflex improves with exercise of the limb
- Proximal muscle wasting
- Ulnar nerve EMG shows increasing amplitude response which decreases in MG
Cervical Myelopathy
Most common cause if Cx spondylosis
Cervical Myelopathy, UMN
= Brisk DTR’s + Spastic weakness Legs
= extensor plantars + ankle clonus
= Lost Vibration and position senses (Rhomberg becomes +)
= Writhing, pseudoathetosis of Hands (with eye closed)
= Rarely spinothalamic sensations lost
= UL biceps, Supinator DTR’s inversion means they are absent but distal finger or Triceps reflex is brisk (increased)
= Myelopathy Hand sign, abducted 5th finger, Rare sphincter issues
= Hand wasting uncommon as supplied by C8T1) by Cx spondylosis affects up
= +_ Lhermitte phenomenon
Proximal Myopathy
Proximal Myopathy causes:
- Polymyalgia rheumatica
- Diabetic amyotrophy
- Cushing syndrome, moon face, acne
- Hypothyroidism, Hyperthyroidism
- Carcinimatous myopathy, small cell lung cancer mostly
- Polymyositis-Dermatomyositi
- McArdle disease - myophosphorylase deficiency
- MERRF syndrome
- Drugs: Alcohol, Steroids, Beta blockers, Amiodarone, INH, Chloroquin, Lithium, Labetolol, Methadone etc
Lateral Popliteal Palsy
Lateral Popliteal Palsy (aka Common Peroneal nerve palsy):
= Foot drop, cannot dorsiflex
= Numbness on lateral side of foot
= Numb patch between 1+2 Toe
= cannot evert foot
———————
Common peroneal nerve has 2 branches - superficial and deep. Superficial peroneal supplies skin on lateral side of foot and peroneus longus & brevis muscles. Deep branch supplies skin between 1 & 2 toe and anterior tibial muscles
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Preservation of inversion of ankle by tibialis posterior distiguishes common peroneal palsy from L4L5 root lesion.
Bulbar Palsy Clinical+Causes
Dysarthria, Dysphagia, S0B
Lesions of 10, 12 cranials
Bulbar Palsy clinical features:
= Absent palatal movements
= Pooling of oral secretions(cant spit out toothpaste)
= Dysarthric indistinct speech
= Wasted atrophic tongue with fasciculations, Nasal twang and aspiration, hangs out
= Nasal regurgitation of food/fluids
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Causes of Bulbar Palsy:
= Motor neuron disease
= Syringobulbia
= Gullain barre syndrome
= Poliomyelitis
= Neurosyphylis
= Base of skull or retropharyngeal pathology
Choreoathetosis
Choreo-athetosis - brief, jerky abrupt quasi-purposeful involuntary mainly facial + UL movements - present at rest & accentuated by activity - gives general air of restlessness.
- Cannot keep tongue protruded, moves in & out.
- Abnormal posturing of hands with wrists flexed, MCP hyperextended and prone when raised
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Causes of chorea:
= Sydenham’s chorea of Rheumatic fev.
= Huntington’s chorea, LL>UL, age >35
= Drugs: L dopa, Neuroleptics
= Senile chorea without dementia
= Rare: Epidemic encephalitis, Wilson’s disease, Hypocalcemia, Thyrotoxicosis, SLE, CO Poisoning,
Hemiballismus
Hemiballismus - flinging wild movements of half side of body, mostly UL - due to stroke or some lesion of sub-thalamic nucleus - disappears during sleep - might wear out in 6-8 weeks
Dystonia: sustained contraction of some body part - usually neck as in Torticollis, Lordosis, Scoliosis
Myoclonus: rapid shock like muscular jerks, often repetitive and may be rhythmic - commonly due to
- epilepsy
- Essential familial myoclonus
- Physiological: stress, exercise, anxiety
- Metabolic(renal, respiratory, Liver)
- Encephalitis
Guillain-Barre syndrome
Guillaine-Barre Syndrome - Acute inflammatory demyelinating polyneuropathy: = usually preceded by URTI or GE due to Campylobacter jejuni in 60% cases
= ascending symmetrical paralysis, foot upwards (LMN) (some has only foot drop but might suddenly worsen)
= absent DTR’s as nerves demyelination
= Bilateral facial weakness, Bulbar weak
= Autonomic: Labile HTN, Tachycardia, Orthostatic Hypotension
= Respiratory paralysis - intubate early, monitor FVC daily
Rx - 1. Plasmapheresis initial 2 weeks or IVIG 2. Monitor Vital capacity, Blood gases, ability to cough and swallow daily 3. Supportive
Miller-Fischer Syndrome
Miller Fischer Syndrome:
= a variation of GBS,
= Anti GQ1b ganglioside antibodies ++,
= External Ophthalmoplegia +
= Ataxia + (as opposed to total paralysis in GBS)
= Areflexia,
= some motor weakness
= High CSF Proteins
= also a AIDP
Ptosis evaluation
Ptosis with
1. Unilateral small pupils - horners
2. Unilateral Dilated pupils - 3rd nerve palsy
3. B/L Normal pupils fatiguability- MG
4. B/ L Normal Pupils with myotonia - MD
Pseudoptosis - thinning of eyelids due to repeated angioneurotic edema
Voluntary Ptosis - to suppress diplopia
Apraxia of the Lids - patient may have to pull down lower eye lids and tilt the head back to open the eyes.
Unilateral Ptosis
Unilateral ptosis causes:
= Horner’s syndrome with meiosis
= 3rd nerve palsy with mydriasis
= Myasthenia gravis, early with 1 eye
= Congenital/Idiopathic - Tensilon -ve
= Myotonic dystrophy: usually B/L
Bilateral Ptosis
Bilateral Ptosis causes
= Myasthenia gravis
= Myotonic dystrophy with other signs
= MERRF or CPEO (chronic progressive external ophthalmoplegia)
= OculoPharyngeal dystrophy (MERRF)
# Kearns Sayre syndrome, (mitochondrial cytopathy with MERRF)
= Progressive ophthalmoplegia
= Retinopathy
= Cardiomyopathy
= Ataxia
Spinal Cord-Root compression
Spinal Cord-Root compression causes
1. Vertebrae and disc lesions
2. Tumors
3. Vasular causes
4. Inflammatory disorders
Spinal Cord-Root Compression-1
- Vertebrae or disc lesions causing it
= Fracture and/or dislocation
= Prolapsed intervertebral disc
= Spondylosis
= Osteoporotic Vertebral collapse
= Paget’s disease
= Atlanto-axial dislocation
= Ankylosing spondylosis
= Sickle cell disease with lysis
Spinal Cord-Root Compression-2
- Tumors causing compression
= Extra-dural: Mets, Lymphoma, Primary Bone tumors
= Intra-dural but extra-medullary: Neurofibroma, Meningioma, Sarcoma
= Intra-medullary: Ependymoma, Astrocytoma, Secondaries
Spinal Cord-Root Compression-3
- Vascular causes of cord compression
= Trauma, haemorrhage
= spinal vascular malformations
= Intra-dural spinal neoplasms
= Coarctation of aorta
= Ruptured spinal artery aneurysm
= Blood dyscrasias
= Anticoagulant causing bleeding
Spinal Cord-Root Compression-4
- inflammatory causes
= Transverse myelitis
= Spinal Osteomyelitis
= TB, Pott’s spine
= Spinal arachnoiditis
= Acute disseminated encephalitis
= Neuromyelitis optica (Devic’s) -autoimmune, affects optic + cord
= Multiple sclerosis
= Progressive necrotising myelopathy
Spinal Cord-Root Compression-clinical
Spinal Cord-Root Compression features
- UMN features- Legs, spastic weakness
- Extensor plantars
- Exaggerated DTR’s or absent if root
- Sensory Levels
- Spinal back pain
- weak plantar flexion in S1 root compr.
Abducence Palsy
Abducence palsy causes
= Mononeuritis multiplex
= Multiple sclerosis
= Any raised ICT cause
= Neoplasm
= Myasthenia gravis
= aneurysmic compression
= Vascular lesions
= Subacute meningitis
clinically- cannot abduct eye, convergent squint, diplopia
Oculomotor nerve Palsy
prominent ophthalmoplegia - intrinsic cause as vascular
Oculomotor Palsy: eye down & out with ptosis + dilated pupil, Unilateral and angulated diplopia
= PCA or IC aneurysm, painful
= Mononeuritis multiplex causes (DM, Vasculitis etc)
= Brainstem stroke
= Multiple sclerosis
= Myasthenia gravis
= Migraine with ophthalmoplegia
= Subacute meningitis
= 3rd N. palsy + contralateral hemiplegia is weber’s syndrome of brainstem stroke.
# Ophthalmoplegia minimal but other features prominent- external compression
Cavernous/ Superior orbital fissure syndrome
Cavernous/Superficial Orbital fissure syndrome -
- total or near total ophthalmoplegia as 3,4, 6 all nerves affected at both locations
- Pain or paraesthesia along V1 division
- absent corneal reflex as V1 involved
causes: Tumor, thrombosis, Carotid aneurysm
Visual Field Defects
- Homonymous hemianopia - vision loss on same side in both eye- say right side - lesion behind optic chiasma
- Bitemporal Hemianopia - vision loss on lateral field of both eyes - lesion at chiasma - pituitary tumor, craniopharyngioma, suprasellar meningioma, aneurysm
- Tunnel vision - central only: RP, Extensive chorioRetinitis, Glaucoma, Hysteria
- Central scotoma -
= with pale optic disc(atrophy),
= swollen pink (papillitis) or
= normal (Retro-bulbar neuritis)
Foster Kennedy Syndrome
Foster Kennedy Syndrome:
= visual loss due to compressive optic atrophy in one eye and papilledema in the contralateral eye brought on by increased intracranial pressure.
= The classical causes of a true Foster-Kennedy Syndrome are frontal lobe, olfactory groove, or sphenoid wing meningiomas.
= The Foster Kennedy syndrome was originally described as the triad of optic atrophy, contralateral papilledema, and anosmia, caused by an olfactory groove meningioma or large frontal lobe tumors. With modern neuroimaging, the diagnosis is often made earlier and anosmia may not be present.
Friedreich Ataxia,
GAA repeats, inherited, Chr-9 AutoRecessive
Early onset, chair bound in teenage
Friedreich Ataxia: degenerative, hereditary, affects dorsal columns, spinocerebellar, optic, CST and cerebellum itself.
- Pes cavus as long standing neuropathy
- Absent Ankle+knee DTR’s+Extensor Plantars
- Ataxia + intention tremor, head shake
- Coarse nystagmus
- Dysarthria - slow slurred or scanning
- Impaired Heel-Shin, Finger-Nose, Rapid alternate hand movements(cerebellar)
- scoliosis
D/d =
1. Tabes Dorsalis (ARP, no deep pain, rhomberg sign+)
2. Multiple sclerosis (brisk DTR, ext Plantars, Optic neuritis history)
3. DM with Cervical spondylosis -absent ankle DTR + ext Plantars
4. Bassen-Kornzweig syndrome
5. Refsum disease
6. Roussy-Levy syndrome
Bassen-Kornzweig Syndrome (Abetalipoproteinemia)
- AutoRecessive
- no LDL, VLDL, Chylomicrons in serum
- very low levels of TG in serum
- Steatorrhoea with malabsorption
- Acanthocytosis
- pigmentary retinal degeneration
- spino-cerebellar degeneration resembling clinically Friedreich’s ataxia
Refsum disease - defective Lipid oxidase
Refsum disease - Phytanic acid accumulation in body due to defective alfa lipid oxidase, AutoRecessive
= Abnormal pupils
= Optic atrophy, Deafness
= Pigmentary retinal degeneration
= Friedreich like ataxia
= Cardiomyopathy
= Icthyosis
Roussy-Levy Syndrome, Variant of HSMN (CMT)
Features of Roussy-Levy syndrome
= demyelinating HSMN, AutoRecessive
= Ataxia like Friedreich
= Areflexia
= Pes cavus
= Upper Limb tremors
= Kyphoscoliosis
= distal limb weakness and atrophy
= clumsiness, Impaired Pain, vibration
Motor Neuron Disease (MND), Types
MND - Types
affects Anterior horn cell i.e. motor spinal nuclei or cranial nerves motor nuclei and has 3 types
1. ALS - Amyotrophic Lateral sclerosis - mixed UMN+LMN involvement with mixed signs, most common, Bulbar weakness can be pre dominant (9, 10)
2. PLS - Primary Lateral Sclerosis - only UMN involvement
3. PMA, Progressive muscular Atropy - only LMN involvement
Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to scarring or hardening (“sclerosis”) in the region.
Motor Neuron disease, Clinical
MND, Clinical features:
= adult onset focal muscle weakness and wasting,
= Muscular fasciculations
= Absent Ankle jerks + extensor plantar
= Bulbar weakness with saliva pooling, aspiration risk, nasal speech
= a weak grip, you might drop things, or find it hard to open jars or do up buttons
= weight loss – your arms or leg muscles may have become thinner over time
= difficulty stopping yourself from crying or laughing in inappropriate situations ie labile emotions
———————————–
always exclude cervical cord compression
MND, Differentials
D/d for MND:
= Cervical cord compression
= Syphilitic amyotrophy
= CNS Lymphoma
= SMA of Juvenile type 3
Multiple sclerosis,
- Relapsing remitting
- Primary Progressive
- Secondary Progressive
Demyelination is believed to be the result of a chronic T cell-mediated, autoimmune inflammatory response against myelin proteins. Thus, MS plaques often are distinguished by gliosis, inflammatory cell infiltration, and localized myelin/oligodendrocyte destruction.
= Fatigue
= Blurred vision, Optic neuritis, pale disc
= Spastic paraplegia (UMN signs)
= Cerebellar signs
= 6th nerve Palsy
= Trigeminal neuralgia
= Pure sensory symptoms
= Uhthoff phenomenon
= Pulfrich phenomenon
= CSF: Proteins rise, Oligoclonal bands
= Visual evoked response delayed
= MRI demyelination plaque + gadolinium enhancement if active inflammation going on
Types of Muscular dystrophy,
Genetic, AD, AR or X linked,
Do Muscle Biopsy, CK levels & Genetics
Myotonic dystrophy – a type of MD that can develop at any age; life expectancy isn’t always affected, but people with a severe form of myotonic dystrophy may have shortened lives
—————————————-
Facioscapulohumeral MD – a type of MD that can develop in childhood or adulthood; it progresses slowly and isn’t usually life-threatening
————————————–
Becker MD – closely related to Duchenne MD, but it develops later in childhood and is less severe; life expectancy isn’t usually affected as much
—————————————–
Limb-girdle MD – a group of conditions that usually develop in late childhood or early adulthood; some variants can progress quickly and be life-threatening, whereas others develop slowly
—————————————
Oculopharyngeal MD – a type of MD that doesn’t usually develop until a person is between 50 and 60 years old, and doesn’t tend to affect life expectancy
—————————————
Emery-Dreifuss MD – a type of MD that develops in childhood or early adulthood; most people with this condition will live until at least middle age
Myotonic dystrophy
Myotonic dystrophy Features
- AutoDom, chromosome 19, both sex
- CTG expansion repeat > 50 - 1000s
- Classic Type 1, or type 2 proximal
- Gradual muscle weakness, foot drop
- Hypogonadism, Percussion myotonia
- Myotonic facies, grip tonic,
- Cataracts, Hypotonia
- Cardiomyopathy
- Myotonic dystrophy type 1(DM1), also called Steinert disease, has a severe congenital form and a milder childhood-onset form.
—————————————
In myotonia congenita, unlike myotonic dystrophy, there is no element of progressive muscular weakness and wasting, cardiac disease, eye abnormalities, endocrine disturbances, or dementia. The life expectancy is the same in patients with myotonia congenita, both dominant and recessive, as in normal individuals.
Localization in Hemiplegia
Hemiplegia + Facial Weakness + Dysphasia (speech):
= Dominant Cortex lesion - precentral (Motor) or Post central gyrus (Sensory)
+++
= Brainstem Signs (nystagmus, ocular palsies, dysarthria, cerebellar signs) - Brainstem lesion or Vertebrobasillar arterial lesion.
Parietal Lobe Signs
Sensory cortex involved so Wernicke’s Dysphasia + Hemiplegia or Hypotonia (rather than spastic) -
+ Apraxias,
+ Agnosias,
+ Dyslexia, Dyscalculia, Dysgrafia,
+ Proprioceptive loss
—————————————
Agnosia = failure to appreciate the sensory impulses even when peripheral mechanisms intact, Can be:
1. Asterognosis/ Tactile: Cannot recognize familial object placed in Hand with eyes closed
2. Visual Agnosia: Cannot recognize object by sight but can by touch or handling it
3. Auditory agnosia: Can recognize the sound of familial object (phone ring, horn, voice, music) only when allowed to touch or see but not by hearing alone (dominant temporal lobe)
4. Autotopagnosia: Inability to recognize anything is wrong - happens in Left hemiplegia in a right handed person - Hemineglect.
Agnosias, a parietal lobe sign
Agnosia = failure to appreciate the sensory impulses even when peripheral mechanisms intact, Can be:
1. Asterognosis/ Tactile: Cannot recognize familial object placed in Hand with eyes closed
2. Visual Agnosia: Cannot recognize object by sight but can by touch or handling it
3. Auditory agnosia: Can recognize the sound of familial object (phone ring, horn, voice, music) only when allowed to touch or see but not by hearing alone (dominant temporal lobe)
4. Autotopagnosia: Inability to recognize anything is wrong - happens in Left hemiplegia in a right handed person - Hemineglect.
Apraxias, parietal lobe sign
Apraxia: cannot execute actions but can recognize the senses. Can be:
1. Dressing apraxia: Affects Upper limbs, cannot dress, use pen, comb or toothbrush
2. Gait apraxia: affects lower limbs, mimics ataxia - unable to lift one foot in front of the other.
3. Truncal apraxia: difficulty in sitting on a chair, turning in bed, getting onto bed
4. Facial Apraxia: cannot use face as in cannot whistle, cannot close eyes, cannot put out his tongue.
LESIONS - can be in Parietal lobe, corpus callosum, pre motor cortex.
5. Constructional apraxia: usually seen with hepatic encephalopathy - person cannot construct simple figure like squares, triangle using match sticks.
Cerebellar Syndrome - Clinical
DANISH
- Dysdidokokinesia - alternate rapid motions lost
- Ataxia to one side or truncal in vermis
- Nystagmus
- Intention tremor, starts with action
- Scanning speech
- ipsilateral Hypotonia of limb with reduced power
= Ipsilateral co-ordination clu)msy(do finger node and heel shin test
————————-
- Pendular knee jerk
- Overshooting of outstretched arm on down
- Skew deviation of eyes: Ipsilateral
goes down & in, contralateral up & Out
- Rhomberg + with eyes open and closed in vermis lesions
-
Cerebellar Syndrome, Causes
Causes of cerebellar lesions
= Stroke, ischemic
= Stroke, Haemorrhagic
= Multiple sclerosis
= Abscess (spread from Otitis, chest etc)
= Alcoholic degeneration
= Brainstem vascular lesions
= Paraneoplastic
= Friedrich ataxia, codon repeats
= CP angle tumors
= Arnold chairi malformations
Spastic paraparesis, Clinical
Spastic paraparesis clinical features
= Lower limb spastic with raised tone
= increased deep tendon reflexes
= Disuse atrophy, contractures if long
= Extensor plantars
= Ankle, Patellar clonus
= Limb weakness
Spastic parapresis, Causes
= Multiple sclerosis
= Cord compression
= Syringomyelia
= Motor neuron disease
= Traumatic cord injury
= Birth injury-Cerebral palsy-Littles disease
= Friedrich ataxia
= ASA thrombosis
= Hereditary spastic paraplegia
= SACD, cord degeneration
= Prasagital cranial meningioma
= HTLV infection (Tropical SP)
= AIDS Myelopathy
= Tabes dorsalis
Clues for cause of Spastic paraparesis
Clues suggestive of cause of SpasticParaparesis
- Cerebellar signs in MS
- Pes cavus in Friedrich ataxia
- Wasted Hand, inverted reflexes in Cx spondylosis
- Horners syndrome in syringomyelia
- Fasciculations with MND
- Absent-ankles+extensor-plantar-SACD
-
HTLV-1/2 associated diseases
Human Tcell Lymphotropic virus
HTLV-1 associated Tropical Spastic paraparesis
HTLV associated Leukemia/ Lymphoma
= High WBC counts
= unexplained hypercalcemia
= Cutaneous lesions
= High HTLV antibodies
HTLV associated polymyositis
HTLV -B-cell chronic lymphocytic leukemia
HTLV associated Infective dermatitis
HTLV-2 + Hairy T cell leukemia
Parkinson’s disease, Causes
Triad of Tremor+Rigidity+Bradykinesia
Causes of Parkinson’s Disease
= Idopathic, commonest
= Drug induced (phenothiazines)
= Post encephalitis Lethargica
= Anoxic Brain damage, Post arrest
= CO Poisoning brain damage
= Manganese toxicity
= Neurosyphillis
= Cerebral tumors affecting basal ganglion
=======================
Other causes with Parkinsonism:
= Vascular Parkinsonism
= Normal pressure Hydrocephalus
= Progressive Supranuclear palsy
= Multiple system atrophy, Striatonigral degeneration
= Cortico-basal degeneration - asterognosis present - cannot recognize coin placed in Hand closed eyes
= Alzeimer’s disease
= Wilson’s disease
= Jakob-Creutzfield disease
= Hypoparathyroidism (BASAL GANGLION CALCIFICATION)
Stiff Person syndrome
Autoimmune association, DM1
Severe progressive muscle stiffness of the spine and lower extremities exacerbated by stress or external stimuli with tendency to fall. Starts 30-50 yr olds, Anti GAD antibodies for DM1 positive in 60%. Other autoimmunes possible as Hypo or hyperthyroidism, Pernicious anemia, Vitiligo.
Progressive Supranuclear Palsy
A supranuclear gaze palsy is an inability to look in a vertical direction as a result of cerebral impairment. There is a loss of the voluntary aspect of eye movements, but, as the brainstem is still intact, all the reflex conjugate eye movements are normal.
= Axial rigidity
= Tendency to fall backward
= Subtle dementia
= Frontal Lobe syndrome
= Parkinsonism
Medications for Parkinson’s disease
Levo dopa based:
Carbidopa/Levodopa 25/100 TID
Carbidopa/Levodopa/Entacapone: 25/100/200 BD
—————–
Dopamine Agonists:
Non Egots:
- Pramipexole 0.125 TID
- Ropirinole 0.25 mg TID
- Rotigotine 2 mg OD
Ergots:
- Bromocriptine 1.25 mg TID
- Pergolide
- Cabergoline
S/q Apomorphine as recuse in crisis
Parkinson’s:
Tremor + Rigidity + Bradykinesia (BRT Parkinson)
Asymmetric in Parkinson’s disease
think from head to toe
- Unblinking expressionless face (Glabellar tap test) can’t blink as bradykinetic
- Titubition (head nodding tremor)
- Blepharoclonus(on gentle eye closure)
- Monotonous speech
- Resting Hand tremor - pill rolling - with synkinesis, increases when check with other action like foot tapping
- bradykinesia: ask to tap thumb with index repeatedely, slow start with fatiguability
- Wrist Cog wheel rigidity
- Elbow Lead pipe rigidity
- Micrographia on writing
- Tendency to fall front or back, cannot balance if pushed
- Stooped posture
- Festinent shuffling gait
Peripheral neuropathy,
distal sensory loss, stocking and Glove, Foot Drop, Hand weakness
Common Causes of PN:
= Diabetes mellitus
= Alcohol and B12 deficiency
= Carcinomatous
= Hereditary Motor Sensory: CMTooth
= Drug induced:
- INH, Ethambutol, Amiodarone
- Metronidazole, Vincristine, Gold,
- Nitrofurantoin, Phenytoin,
- Hydralazine, Chloramphenicol,
- Cyclosporine
Rare Causes of PN
Rare Causes of PN
= Guillain Barre syndrome
= Polyarteritis nodosa
= Rheumatoid arthritis, CTD’s
= Amyloidosis, thick nerves
= AIDS
= Chronic IDP
—————————————-
= Myxoedema
= Acromegaly
= Sarcoidosis
= Uremia
= Lyme disease
= Tetanus
= Botulism
= Paraproteinemia
= Refsum’s disease
= Arsenic poisoning
= -Tri-ortho-cresyl phosphate poisoning
Prominent Motor PN
Causes of Predominantly motor PN
= Carcinomatous
= Lead induced
= Porphyria
= Diphtheria
= CMT disease
POEMS Syndrome (with myeloma)
Osteosclerotic myeloma associated
- Polyneuropathy
- Organomegaly - HepatoSplenomegaly, Lymphadenopathy
- Endocrinopathy- DM, Hypothyroidism, Hypogonadism
- Monoclonal gammopathy, plasma cell disorder, IgA, IgG
- Skin changes - melanoderma,buccal fat pad atrophy,
High levels of Vasc endothelial growth factor ( VEGF)
Rx - Radiotherapy, Melphalan, Thalidomide
Multiple System Atrophy
Multiple system atrophy (MSA) is a rare condition of the nervous system that causes gradual damage to nerve cells in the brain. This affects balance, movement and the autonomic nervous system, which controls several basic functions, such as breathing, digestion and bladder control.
What is cortico-basal degeneration
Corticobasal degeneration (CBD) is a form of frontotemporal degeneration, a dementia that involves the loss of cognitive functions such as the ability to think, remember, or reason to the point that it interferes with a person’s daily life and activities. CBD can affect: Movement. Balance
Painless Neck Weakness
= Myasthenia gravis
= amyotic Dystrophy
= Polymyositis
= Motor neuron disease
Rinne test, Hearing
The Rinne test is performed by placing a 512 Hz vibrating tuning fork against the patient’s mastoid bone and asking the patient to tell you when the sound is no longer heard. Once the patient signals they can’t hear it, the still vibrating tuning fork is then placed 1–2 cm from the auditory canal.
The Rinne test is used when conductive hearing loss is suspected and used in patients with otosclerosis to determine if a patient might benefit from stapes surgery.
The patient should be able to hear the sound of the tuning fork adjacent to their ear, persist for approximately twice as long as the sound they heard over their mastoid process. This is considered a “positive test means normal (air > bone)
Weber test, Hearing
The Weber test has been mainly used to establish a diagnosis in patients with unilateral hearing loss to distinguish between conductive and sensorineural hearing loss
Place the base of a struck tuning fork on the bridge of the forehead, nose, or teeth. In a normal test, there is no lateralization of sound. With unilateral conductive loss, sound lateralizes toward affected ear. With unilateral sensorineural loss, sound lateralizes to the normal or better-hearing side.
Neuromyelitis Optica
Devic’s disease
Neuromyelitis optica (NMO), (Devic’s disease) is a rare autoimmune, more common in women > men, can have-
= Pain in the eyes (Optic neuritis)
= Loss of vision.
= Weakness or numbness or paralysis in the arms and legs.
= Difficulty controlling the bladder or bowels.
= Uncontrollable vomiting and hiccups.
to diagnose NMO is testing your blood for AQP4 or MOG antibodies. While blood testing can’t always confirm NMO — because about 13.5% of cases don’t involve identifiable antibodies.
MRI required.
Eculizumab is a complement inhibitor of protein C5, preventing the cleavage into C5a and C5b. Eculizumab was the first monoclonal antibody FDA approved for the treatment of NMO/NMOSD in adult patients who are seropositive for AQP-4 and may be considered a first-line treatment option.
McDonald Criteria for Multiple Sclerosis diagnosis,
(MRI based)
T2 MRI lesions are the white spots observed on MRI using the typical imaging sequences acquired to diagnose and monitor Multiple Sclerosis. The most popular of these sequences is a FLAIR image (Fluid Attenuated Inversion Recovery).
Criteria
The diagnosis of multiple sclerosis can be made if there is fulfillment of any of these five categories of criteria, depending on how many clinical attacks have occurred:
≥2 clinical attacks with ≥2 lesions with objective clinical evidence with no additional data needed
≥2 clinical attacks
with 1 lesion with objective clinical evidence and a clinical history suggestive of a previous lesion with no additional dta needed
≥2 clinical attacks with 1 lesion with objective clinical evidence and no clinical history suggestive of a previous lesion
with dissemination in space evident on MRI
1 clinical attack (i.e. clinically isolated syndrome) with ≥2 lesions with objective clinical evidence with dissemination in time evident on MRI or demonstration of CSF-specific oligoclonal bands
1 clinical attack (i.e. clinically isolated syndrome) with 1 lesion with objective clinical evidence with dissemination in space evident on MRI with dissemination in time evident on MRI or demonstration of CSF-specific oligoclonal bands
Dissemination in space
Dissemination in space requires ≥1 T2-hyperintense lesions (≥3 mm in long axis), symptomatic and/or asymptomatic, that are characteristic of multiple sclerosis in two or more of the four following locations:
periventricular (≥1 lesion, unless the patient is over the age of 50 in which case it is advised to seek a higher number of lesions)
- cortical or juxtacortical (≥1 lesion)
- infratentorial (≥1 lesion)
- spinal cord (≥1 lesion)
Notably, T2-hyperintense lesions of the optic nerve, such as those in a patient presenting with optic neuritis, cannot be used in fulfilling the 2017 revised McDonald criteria 5.
Dissemination in time
Dissemination in time can be established in one of two ways:
- a new T2-hyperintense or gadolinium-enhancing lesion when compared to a previous baseline MRI scan (irrespective of timing)
- simultaneous presence of a gadolinium-enhancing lesion and a non-enhancing T2-hyperintense lesion on any one MRI scan
Primary progressive multiple sclerosis (PPMS)- In addition to the above criteria, the McDonald criteria also defines the diagnosis of primary progressive multiple sclerosis. The diagnosis now requires:
≥1 year of disability progression which can be determined either prospectively or retrospectively
with two of the following:
≥1 T2-hyperintense lesions characteristic of multiple sclerosis in one or more of the following regions: periventricular, cortical or juxtacortical, or infratentorial
≥2 T2-hyperintense lesions in the spinal cord
presence of CSF-specific oligoclonal bands
Multiple Sclerosis Treatment Options
Acute Relapse: to shorten it
1. High dose Methyl Prednisolone, 0.5 to 1 Gram IV OD for 3-5 Days
2. Plasma exchange, 5 times if above fails
3. IV IG
Prevention of New Lesions or further relapse:
= Interferon B1a
= Interferon B1b
= Natalizumab (Tysabri) infusion
= Glatiramer acetate (copaxone)
= Mitoxantrone (Novantrone)
= Fingolimod (Gilyena), oral agent
Symptomatic therapies:
= for neurogenic bladder: 1. Anticholinergics: Oxybutinin, Tolterodine,
2. Intermittent Self Cathterization
3. Urology referral
4. Botulinum toxin injections
= for spasticity: Baclofen, Valium, Tizanidine, Botulinum
= Neuropathic analgesics
= Diet, Peg Tubes
= Anti depressants, Behavioral therapy
= Offer MS society help
MS treatment
1. Interferon beta use criteria
2. Natalizumab use criteria
Criteria for Use of Interferon Beta:
= > 2 clinical relapses in last 2 years
= able to walk 10 m or more
= not pregnant or attempting it
= > 18 yrs and no contra indications to use of interferon
Criteria for Use of Natalizumab for MS:
= for rapidly evolving severe disease
= > 2 relapses in 1 year
= > 1 Gadolinum enhancing lesions MRI
or
significant increase in T2 lesion load as compared to past MRI
Natalizumab, sold under the brand name Tysabri among others, is a medication used to treat multiple sclerosis and Crohn’s disease. It is a humanized monoclonal antibody against the cell adhesion molecule α4-integrin. It is given by intravenous infusion every 28 days. (company- Biogen idec) - can cause PML and if stopped - immune reconstitution syndrome.
CTS differentials
= Brachial plexopathy
= Peripheral neuropathy
= Mononeuritis multiplex
= Reflex sympathetic dystrophy, Post trauma to nerve
= C6C7 or C8T1 root radiculopathy
CTS association, underlying cause
= Hypothyroidism
= Amylodosis
= Rheumatoid arthritis
= Cushing’s syndrome
= Acromegaly
= Distal radius bone fracture
= Obesity
= Dialysis patient
= Pregnancy or Menopause
= Vibrating tools Job
Anterior interosseus nerve, a branch of Median nerve in proximal forearm
The anterior interosseous nerve supplies flexor pollicis longus & radial part of flexor digitorum profundus. This branch runs on the interosseous membrane with anterior interosseous artery passes deep to the pronator quadratus & supplies it. It terminates by giving articular branches to the distal radio ulnar, radiocarpal & carpal joints.
It also supplies to proximal part of the palm, via the palmar cutaneous branch. This branch does not enter the carpal tunnel and is hence spared in carpal tunnel syndrome. Loss of sensations over thenar skin implies proximal lesion in forearm. weakness of thumb and index finger to form a tight circles is found in pronator teres syndrome.
Tremor characteristics
- Resting tremor - in Parkinson’s
- Tremor with changes in posture - postural tremor
- Movement or while approaching target - intention tremor in Cerebellar disease
- All time tremor - drugs/Metabolic/Physiological
- Eased by Alcohol - essential tremor
With Double vision - PSP with parkinson
With Dysarthria/Salivation/Dysphagia
With Hand cramps/ neck cramps - dystonia, torticollis
With Postural Dizziness/ Sphinter disturbance/Palpitations (Autonomics) - seen in Shy Drager with parkinsonism
With Dementia - Lewy Body dementia with Parkinsonism
Drugs causing Tremors
- beta agonists, Salbutamol/ Terbutaline/ Aminophylline etc
- Thyroxine
- SSRI, TCA’s
- Caffeine
- Valproate, Lithium etc
- Alcohol withdrawal
- Valium withdrawal
- Morphine withdrawal
Manganese, Mercury exposure can cause neuro tremors, Parkinsons etc
Diplopia: Questions to ask
Weight loss + Night sweats + Head ache increasing on cough - CNS SOL
= which eye, mono or binocular
= Which direction of gaze, descending stairs (SO4), lateral gaze (LR6), all time (3rd nerve)
= Blurring or loss of vision
= Pain in and around eye
= any past same episodes
= Fatiguability: improves after resting/sleeping for few Hours? - MG/ inflammatory, GBS??
OR worse when trying to focus as watching TV.
non eye symptoms:
= any associated headache/ speech problems/ hearing issues/ ringing in ears/ vertigo/ nausea/ vomiting (brainstem)
= if Headache: which part, lying more or standing? coughing/ sneezing ?
= any sensory-motor limb issues?
Acute Diplopia Examination
Assess speech - 1 & 2 step commands
Assess orientation in time/place/person
All cranial nerves:
= Visual acuity
= Ptosis
= Pupils, Light, Direct/Consensual
= Visual Fields, each eye (CVA)
= blind spot assessment
= Eye movements for 3/4/6, fatiguability for Thyrotoxicosis/ MG/ Miller fischer/GBS/ Vascular
= Saccades, palm/ fist: slow in Brainstem diseases
= Nystagmus or INO with MS
= Fundus: Papilloedema, DM changes
———————–
TEMPORAL ARTERY TENDERNESS
RHOMBERG TEST
CEREBELLAR SIGNS
PULSE FOR Afib
Blood pressure both hands
Carotid Bruit, Heart Murmurs
Limb signs for stroke
Acute Diplopia, causes
Mostly vascular, inflammatory if acute
Otherwise also - Autoimmune, SOL, Infiltrative
All cases - stroke work up required
Autoimmune: CTD’s like RA, SLE, Sjogren’s, Behcet;s, MG, GBS
Vascular: microvascular (DM), thromboembolic (AF), Superior orbital fissure syndrome, Right Cavernous sinus lesions, MCA aneurysm
Infiltrative: Amyloidosis, Sarcoidosis
Infections: Lyme, TB, HIV, HTLV,
Other: Thyroid, Wernickes, CNS tumors as CP angle, Lymphoma, Posterior fossa
Pakinson’s disease Symptoms
3/5 = Bradykinesia, Tremor, Rigidity, Postural instabilty, Gait disorder
Early - Day sleeping, Loss of smell
- Asymmetrical tremor, difficulty with - hand tasks as buttoning
- Walking/ turning difficulty, Reduced speed, Initial freezing then festiant Gait, Loss of arm swing and Falls
- Micrographia
- Poor facial expression
- Associated symptoms:
= constipation
= Fatigue, poor sleep
= Drooling of saliva
think of parkinson’s plus syndromes
Parkinson’s plus syndrome
These are ones with parkinsonian symptoms/ signs but usualy symmentrical and plus issues
? Visual hallucinations: lewy body dementia with Parkinsonism (not PD)
? Postural Hypotension or Autonomic symptoms as palpitations, sweating,
bladder dysfunction - MSA, Shy Drager
? Difficulty in looking up ? Progressive supra nuclear palsy with Parkinsonism
? Vascular risk factors like smoking/ Strokes/ MI/ DM - Vascular Parkinsonism
Parkinson’s Signs
Mask like, expressionless face
Hypersalivation at angle of mouth
Reduced blinking - glabellar tap test
Unilateral Hand tremor, 4-6 hrtz, pill roll
Cog wheel rigidity at risk
Lead pipe rigidity at elbow
Check tone while patient tapping other hand on to bed (activation phenomenon) which exacerbates tone
Ask to write - micrographia
Bradykinesia: ask to tap thumb-index fingers onto each other rapidly
and rapidly supinate, pronate hand
Ask to read some thing - soft voice and no tones
Postural inastabilty check:
Retropulsion (pull suddenly towards you)
Gait: small shuffling, turning en block
Other for Plus syndromes:
- Supine and standing bp
- Upwards gaze in pursuit lost in PSP but same vertical doll’s eye is not lost
-
Parkinson’s Differential
- Idiopathic when called P. Disease
- Drug induced: Antiemetics, Neuroleptics (phenothiazines, thioxanthenes, butyrophenones, Clozapine and risperidone)
- Vascular-Parkinson: Sticky feet +
- PSP: Vertical gaze palsy, truncal instability
- MSA - Postural Hypotension (Autonomic failure, Cerebellar ataxia)
- Diffuse Lewy Body dementia: with visual hallucinations
- Essential Tremor, improves by Alcohol, intention tremor rather than resting
Pure Autonomic Failure
The main symptom of PAF is orthostatic hypotension, a sudden drop in blood pressure upon standing. This can cause dizziness, lightheadedness, blurry vision, and weakness. Other symptoms can include fatigue, bladder problems, constipation, abnormal sweating, and sleep disorders.
Pure autonomic failure is caused by abnormal accumulation of synuclein in the brain. Blood pressure may decrease when people stand, and they may sweat less and may have eye problems, retain urine, become constipated, or lose control of bowel movements.
Stroke in Young Patients
- Antiphospholipid syndrome
- Vasculitis: SLE, Sjogrens
- VHD with emboiism or Afib
- Aneurysm rupture
- Hyperlipidemia
- complex Hemiplegic migraine
- Carotid dissection - neck extension at Hair dresser’s or looking up or trauma, comes with neck pain and Horner’s syndrome
- Vertebral dissection - brainstem symptoms, same mechanism as carotid
- Paradoxical embolism with ASD/ PFO
OC pill use and Migraine are independant risk factors - suggest alternate contraception as IUD/Progrestogen only pill or implant, Barrier method
Migraine: clinical features
- Unilateral Headache, usually
- Aura(visual/ sensory/ auditory/ motor), blurring of vision, Fortification spectra (Fortification illusion specifically refers to a visual hallucination where geometric lines arrange themselves in something that many say looks like a castle fortification,” )
- Nausea, Photphobia
- Hemiplegia, transient
- Common Triggers: Chocolate, Cheese, Sleep deprivation, Starvation, Around menses, Caffeine, Alcohol, Exercise
- Relief with sleeping in quiet dark room
Carotid Dissection Presentation
- sudden neck pain after hyper-extention as at hairdresser or trying to take some thing off top shelf or painting a ceiling looking up
- Horner’s: Meiosis+Ptosis+forehead sweating loss
- Los of taste
- Amaurosis fugax
- Focal limb weakness some times
- Pulsatile tinnitus or audible bruit
- Neck swelling like hematoma
- Migraine like symptoms
- TIA or stroke like symptoms rarely due to broken off clot
Transient Loss of Vision, Differential
= TIA, Amaurosis fugax ( Greek “amaurosis,” meaning dark, and the Latin “fugax,” meaning fleeting)
= Acephalgic Migraine, young women
= Cardio-embolic, Afib, LV thrombi, IE
= Local eye causes - vitreous H’rrhage
= Optic neuritis
= High Stroke risk factors (ABCD2 score)
ABCD2 score
Admit/ Discharge in case of TIA
Score > 4 = review within 24 Hrs
Score 0-3 = TIA clinic within 7 days
4 weeks no driving
Age > 60 = 01 point
BP @ presentation > 140/90 = 01
Clinical:
# weakness = 02
# Speech disturbance no weakness 01
Duration of TIA
# > 60 min = 02
# < 60 min =01
Diabetes mellitus = 01
Admit if:
Crescendo TIA, 2 or more in 7 days
Lateralizing signs on examination
Symptoms still present on arrival
Fluctuating symptoms
Early specialist Review < 24 Hrs if:
ABCD2 score > 4
Chronic or Paroxysmal Afib
on Warfarin
With Prosthetic valve
Young, age < 50 yrs
Thunderclap Headache, Differentials
= Subarachnoid headache, mostly aneurysmal bleed in young patients
= Cerebral sinus thrombosis
= Arterial dissection
= Colloid cyst of 3rd ventricle
= Reversible Cerebral Vasoconstriction syndrome
= Early meningitis( rash, neck stiffness, Photophobia)
= Blitz Migraine
= Cough Headache
= Coital Cephalgia
Upper Limb Joint Nerve Roots
Shoulder:
= Abduction C4C5
= Adduction C6C7
Elbow:
= Flexion C5C6
= Extension C7C8
= Pronation C6
= Supination C6
Wrist:
= Flexion C7C8
= Extension C7C8
Fingers:
= Flexion C7C8
= Extension C7C8
= Adduction C7
= Abduction C7
Dementia Differential
= Alzeimer’s disease
= Fronto-Temporal dementia
= Hutington’s chorea
= Dementia with Lewy bodies
= Vascular Dementia (multiple strokes)
= Leuco encephalopathies
= Parkinson’s disease associated
Astereognosis
Unable to recognize the objects by touch alone as a key in Hand.
Happens with Parietal lobe SOL and cortico-basal degeneration.
Anosmia Causes
= Viral URTI, sinusitis, HEAD INJURY
= Idiopathic Parkinsons disease
= Alzeimer’s disease
= Olfactory groove meningioma
= Superficial siderosis
= Kallman’s syndrome: Anosmia+Hypogonadism+Xlinked Recessive
= Refsum’s disease
= Paraneoplastic disorders
Autonomic Storm
Massive catecholamine surge with rise of BP, Pulse and temperature.
= Post CPR anoxia
= Toxins and drugs can cause it:
= PhenyL propanolamine
= Cocaine
= Amphetamines
= TCA’s
= Botulism
= Tetanus
= Neuroleptics
= SAH, Stroke, Tumors, Hydrocephalus:
Rx: ICU, Supportive, Morphine, Labetolol
TESTS for Autonomic System
HRDB: Heart Rate Response to Deep Breath,
Valsalva Ratio: persistent expiratory pressure 40 mmHgfor 10-15 seconds. Ratio is maximum tachycardia in phase 2 to maximum bradycardia in stage 4.
QSART: Quantitative Sudomotor axon Reflex Test
Beat to beat BP response to Valsalva
HUT: Head up tilt test
Autonomic Neuropathies
Acute Auto-Neuropathy
= Subacute Autoimmune
= Paraneoplastic auto-neuropathy
= GBS (AIDP)
= Botulism
= Porphyria
= Drug induced AN
= Toxic AN
Chronic Auto-Neuropathy:
= Distal small fiber AN
= Combined sympathetic and paraSympathetic AN seen with:
Amyloidosis
DM associated
Autoimmune
Paraneoplastic
Familial (Riley Day syn)
Sensory Neuropathy
Gerstmann Syndrome
Dominant parietal lobe lesion
Dysphasia: Receptive/Expressive/Global
Dysgraphia: deficiency in ability to write coherently
Dyslexia: trouble with reading despite normal intelligence
Dyscalculia: difficulty with calculations
Finger agnosia: difficult in recognizing finger names or on touch or name then own as well as of others
Chronic myelopathies
Chronic myelopathy of MS
SACD, Tabes Dorsalis
Familial spastic paraplegia
Adrenomyeloneuropathy
Primary Lateral Sclerosis
Lathyrism: Grass Pea consumption
NO inhalation associated
Radiation associated
Compressive or Metastasis related
SLE, Sjogren’s Sarcoidosis
Ring Enhancing Brain Lesions
Large Brain Metastasis
Toxoplasmosis
CNS lymphomas
Brain abscesses
Radiation necrosis
Granulomas
Tuberculosis lesions
Sarcoidosis lesions
Demyelination lesions
Always consider Stroke, ICH, Trauma as differentials in all REL’s.
One and Half syndrome
It presents a combination of ipsilateral conjugate horizontal gaze palsy (one) and ipsilateral internuclear ophthalmoplegia (INO) (a half)
One-and-a-half syndrome is most often caused by multiple sclerosis (MS), brain stem stroke, brain stem tumors, and arteriovenous malformations.J
The location of the lesion is the paramedian pontine reticular formation or VI nerve nucleus.
Eight-and-a-half syndrome
Eight-and-a-half (EHS) syndrome is a rare neuro-ophthalmologic condition which defines as one-and-a-half syndrome plus facial nerve palsy ipsilateral to the side of one eye which has no movement (1.5 + 7 = 8.5).
Cavernous sinus syndrome
Both Cavernous sinuses connects each other.
Cavernous sinus syndrome is characterized by ophthalmoplegia and sensory deficits over the head due to combined deficits of the three cranial nerves (third, fourth, and sixth) responsible for eye movements and pupil function, and at least one branch of the trigeminal nerve. It is Life threatening and may have fever.
Suspect if sudden peri orbital swelling and edema both sides.
= CV cellulitis: sinus to facial to ophthalmic vein spread.
= Mucormycosis in DM or on steroids
= Carotid Cavernous fistula
= Carotid Artery aneurysm
= Meningioma
= Nasopharyngeal cancer
= Idiopathic granulomatous-Tolosa Hunt sydrome
Trigeminal Neuropathy,
Causes and location of lesions
- Nuclear Lesion - Brainstem, Upper cord: MS, Stroke, Syringobulbia, Gliomas, Lymphomas
- Pre-Ganglionic Lesions: Acoustic neuroma, Meningioma, Mets, Chronic meningitis, Cavernous-Carotid aneurysm
- Gasserian Ganglion Lesions: Trigeminal neuroma, Herpes zoster, Infection spread from Otitis media/ mastoiditis
- Trigeminal nerve lesions: Ca nasopharynx, Trauma, GBS, Sjogrens, CTD’s, Sarcoidosis, Idiopathic
Certain drugs: Stillbamidine, Trichloroethyline
Glosso-pharyngeal neuralgia
- similar to trigeminal neuralgia
- Shooting pain across throat, tonsils
can spread to ears due to tympanic branch - Vagus involvement can cause cardiac arrhythmias, asystole, fainting attacks
- Look for zoster lesions in ears (hidden)
Treatment:
- Pain control with Carbamazepine/ Pregabalin/ Gabapentin
- Micro vascular decompression
- Rhizotomy of 9, 10th n. in Jugular bulb
Palinopsia
Palinopsia: persistent images once the stimulus has been removed from visual field. Seen with occipital lobe SOLs
Polyopia: seeing multiple images
P1 syndromes,
P1 segment branch of PCA
affects: Midbrain, Subthalamic and thalamic areas
Claude’s syn: 3rd palsy + contralateral ataxia, red nucleus affected
Webers: 3rd palsy + contra hemiplegia as cerebral peduncles affected
Occlusion of Artery of Percheron: paresis of upward gaze + abulia ie slowness of response + drowsiness
Dejerine Roussy syn: thalamic infarcts, contralateral sensory loss of pain + then later severe pain without stimulus or with minor one (neuropathic)
Both PCA occlusion: Coma, Unreactive pupils, decerebrate rigidity, Bilateral pyramidal signs (extensor plantars, hemiplagia, spasticity, brisk DTR)
Cerebellar syndromes
DASHING & PASTRIES
The arterial: Vertebro-basilar system: superior cerebellar artery (SCA), the anterior and inferior cerebellar artery (AICA), and the posterior and inferior cerebellar artery (PICA).
Causes of Cerebellar Syndromes:
P-araneoplastic- ca lung
A-lcohol
S-clerosis multiple-MS
T-umor, SOLs
R-are: Friedrich, ataxia telangiectasia
I-diopathic
I-atrogenic: drugs: Phenytoin
E-ndocrines: Hypothyroidism
S-trokes - PICA/SCA/AICA
Autonomic Disorders
Brain - Spinal - Neuropathy
- Autonomic-Brain origin:
with MSA: - Prominant Autonomic:
= MSA
= Parkinson’s + Autonomic failure
= Dementia with Lewy bodies - Autonomic not prominant + MSA
= Parkinson’s disease
= Progressive Supranuclear palsy
= Cortico-basal degeneration
= Machado-Josphine syndrome
without MSA:
1. Mainly cortical - frontal, Parietal
2. Limbic/ ParaLimbic:
= Shapiro Syndrome
= Autonomic seizures
3. Hypothalamic disorders
4. Brainstem/Cerebellar: Posterior fossa SOLs, Arnold Chairi malformation, syringobulbia
Chorea, (means dance), causes
ST VITUS DANCE
S= Sydenhams (in RHEUMATIC fever)
V= Vascular strokes
I= Increased RBC’s (polycythemia)
T= Toxins: CO, Mg, Hg
U= Uremia
S= SLE, MOST COMMON in adults
S= enile chorea
D= rugs
A= Anti Phospho Lipid syndrome
N= Neurodegenerative: Huntingtons, neuro-acanthocytosis, DRPLA
C= ConceptionS: pregnancy, OCP’s
E= Endocrine: hyperthyroidism, hypo-, hyperglycemia
Neuro-Acanthocytosis
Neuroacanthocytosis refers to a group of genetic conditions that are characterized by movement disorders and acanthocytosis (abnormal, spiculated or star-shaped red blood cells).
Four syndromes are classified as neuroacanthocytosis:
1. Chorea-acanthocytosis
2. McLeod syndrome
3. Huntington’s disease-like 2 (HDL2)
4. Panthothenate kinase-associated neurodegeneration (PKAN)
Symptoms and Signs include:
Chorea (involuntary, dance-like)
Parkinsonism (slowness of movement)
Dystonia (abnormal body postures)
Problems walking
Muscle weakness
Involuntary movements of the face and tongue
Tongue/lip biting (which is mostly characteristic of chorea-acanthocytosis)
Difficulty with speech and eating
Cognitive impairment
Psychiatric symptoms - self mutilation
Seizures
Cardiac problems in McLeods
Mini Mental Status Examination (MMSE), 30 point score
Orientation:
- Name season/Day/Date/Month/Yr
- Name Hospital/Town/State/Country
Registration: Tell 3 common objects names and repeat them
Attention Calculation: substract 7 from 100 upto 5
Recall: 3 objects told earlier at 5 min and 15 minute recall
Language: Name pencil, watch (what is it?)
Repeat: no ifs, ands or buts
3 step commands: ask to 1. take this paper 2. fold it in half and 3. put it on table
Written commands: ask to read and do - close your eye now
Write a sentence - ask to write a sentence by themselves
Copy a design - intersecting pentagrams or a clock
Treatment of Dystonias
Levodopa
Trihexphenydil
Oral Baclofen
Intra-thecal baclofen
Tetrabenazine (12.5 to 200 mg/Day)
Botulinum injections
DBS, bilaterally for Parkinsons
Dystonic storm: Focal or multi focal dystonia during stress - vocal cord dystonia, Airway obstruction and rhabdomyolysis
Dystonic Storm
Dystonic storm is a frightening hyperkinetic movement disorder emergency.
Marked, rapid exacerbation of dystonia requires prompt intervention & ICU admission. Clinical features of dystonic storm include: = fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failure.
Dystonic storm usually occurs in patients with known dystonia, such as DYT1 dystonia, Wilson’s disease and dystonic cerebral palsy.
Triggers such as infection or medication adjustment are present in about one-third of all events.
Rx = DBS, IV Baclofen, supportive otherwise
Dystonia Types
Dystonias are involuntary, sustained, repetitive muscle contractions.
1. Primary dystonia:
= Idiopathic Torsional (Oppenheim’s) - childhood onset, AutoDom,
= Dopa Responsive (Segawa’s): childhood, AutoDom, defective tyrosine hydroxylase, improves with sleep, foot dystonia on walking,
= Focal Dystonias: Blephalospasm, Torticollis, Oromandibular, Spasmodic dysphonia, Limb dystonias (writers cramps, Musicians)
2. Secondary Dystonia:
= Drug induced (metoclopramide)
= Toxins : CO, Mg, Hg
= Part of neurological illness
HIV associated Neuropathies
= Distal symmetric polyneuropathy: Nigh time painful paraesthesia, distal sensory loss, absent ankle DTRs
= GBS, CIDP (demyelinating)
= Mononeuropathy multiplex: multiple seperate nerves affected, foot drop, wrist drop, focal weakness (HCV, Cryoglobiulinemia)
= Polyradiculopathy with CMV: flaccid paraparesis, saddle numbness, Urine retention
= Toxic Neuropathy of HAART: with stavadine, didanosine, zalcitabione (need to stop them)
= Focal with Zoster/ Lymphoma/ TB
Vitamin E deficiency
caused by irregularities in dietary fat absorption or metabolism. Vitamin E is a lipid-soluble nutrient. A normal level is usually with the range of 5.5–17 milligrams per liter (mg/L).
Disorders that cause fat malabsorption, including abetalipoproteinemia (Bassen-Kornzweig syndrome, due to genetic absence of apolipoprotein B), chronic cholestatic hepatobiliary disease, pancreatitis, short bowel syndrome, and cystic fibrosis
A rare genetic form of vitamin E deficiency without fat malabsorption results from defective liver metabolism.
resembles friendrich ataxia
ataxias, distal wasting, pes cavus, proximal weakness chair bound
Vision loss
Test Serum Tocopherol levels
Test Serum Tcopherol: Lipid ratio
Rx Alfa Tocopherol 400 mg BD
Dry Beri Beri (Thiamine low), B1
Thiamine was named by the Williams team as a portmanteau of “thio” (meaning sulfur-containing) and “vitamin”. The term “vitamin” coming indirectly, by way of Funk, from the amine group of thiamine itself (although by this time, vitamins were known to not always be amines, for example, vitamin C). Thiamine was also synthesized by the Williams group in 1936.
Stocking and glove sensory loss, Distal Leg weakness with ataxia, Lost ankle DTR.
in Alcoholics, anorexics, Hyperemesis, Starvation,
Do - thiamine check, NCV, Sural biopsy,
RBC trans-kerolase activity low
Rx Thiamine IV & oral 100 mg OD
Peripheral Neuropathy causes
Metabolic: DM, CKD, Porphyrias, Amyloidosis
Infections: HIV, Lyme, Syphillis, CMV, Leprosy, Diphtheria
Nutritional: B12, B1, B6 deficiency
Immunological: GBS, CIDP, Multi-focal motor, Anti-MAG-neuropathy
Paraneoplastic: Ca Lung
Toxic: Alcohol, HAART, Platinum drugs, Amiodarone, Tick bites, INH, Phenytoin
Hereditary: Charcot-Marie-Tooth (HMSN)
Vasculitic: PAN, Churg-Strauss,
Cryoglobulinemia in HCV
Odd: Coeliac, Hypothyroidism, Fabry’s disease
Neuropathies, Histolgical
- Demyelinating: mostly proximal, acute or subacute, paraesthesias, weakness, vibration/Positon loss more than pain and temp, areflexia
NCV shows velocity > amplitude, demyelination and then remyelination with rapid recovery = GBS, CIDP, Diphtheria, DM, Multifocal Motor neuropathy - Axonal : distal weakness more than proximal, slow progression, Pain/temp loss more than vibration/Position, paraesthesias, DTR loss,
NCV shows: amplitude > velocity.
Axon degenerates and regenerates
Slow recovery, examples - toxic, metabolic, HIV related, DM, HMSN - Neuronal:non length dependant, rapid paraesthesia, ataxic gait, vibration/ position loss more than pain/temp, areflexia, proprioceptive weakness, rhomberg +
NCV: sensory amplitude
poor recovery, no regeneration
examples: Cisplatin related, Sjogren’s related
Peripheral neuropathies by Progression/ Time course
Acute: GBS, Toxic, Diphtheric, Thallium related, TriOrthocresyl phosphate (an Organophosphate flame retardant)
Sub-Acute: Toxic (Hexacarbon, acrylamide, Angiopathic, Nutritional, Alcoholic
Chronic: DM, CIDP, Paraneoplastic, Paraprotenemic,
Long Standing: Hereditary (CMTD) Friedrich
Recurrent: Relapsing CIDP, Porphyria, Refsum’s disease, Hereditary neuropathy with pressure palsies
Entrapment neuropathies as in pagets
Treatment of Polyneuropathies
General: Education, Foot care, Physiotherapy, offer Support as Social and occupation referral
Pain control:
Amitryptiline, Dulaxetine, Imipramine
Gabapentin/Pregabalin/Mazetol as 2ndline
Oral Morphine sulfate 3rd line
Oxycontin 5 mg BD, Epidural analgesia
Buprenorphine 35 mcg patch
Topiramate 25 mg BD
Clonazepam HS
Treat Systemic Cause
IVIG for CIDP, Mutifocal Motor neuropathy
CST, Plasma Exchange with CIPD
Peripheral Neuropathy by Fiber type
Large fiber neuropathy manifests with the loss of joint position and vibration sense and sensory ataxia, whereas small fiber neuropathy manifests with the impairment of pain, temperature and autonomic functions.
Small Fiber sensory:
painful, dissociated sensory loss = HSMN, Lepromatous leprosy, DM, Amyloidosis, alfalipoproteinemia, Fabry’s disease, HIV etc
Large Fiber Sensory:
ataxic, = Sjogren’s syndrome, Friedrich ataxia, B12 deficiency, Cisplatin, Pyridoxine toxicity
Small + Large Fibers:
Global sensory loss,
carcinomatous, Hereditary, DM, Xanthomatous neuropathy in PBC
Vacor ingestion
Motor Predominant:
GBS, AIDP, Diphtheric, HSMN, Lead, Brachial neuritis, DM Amyotrophy
Autonomic:
Botulism, GBS, Porphyria, Vacor, Amiodarone, Vincristine,
Chronically with DM, Amyloidosis, Sjogren’s, HSAN, Chagas
Vacor Ingestion Neuropathy
Ingestion of Vacor (a rodenticide) results in a rapidly progressive, severe, distal axonopathy with accompanying diabetes mellitus, autonomic dysfunction, and encephalopathy. Diabetes is due to necrosis of pancreatic beta cells.
A clinical syndrome, characterized by acute diabetic ketoacidosis associated with a toxic neuropathy, developed in five men who intentionally ingested a recently introduced rodenticide (Vacor) containing N-3-pyridylmethyl-N’-p-nitrophenyl urea (RH-787). A 7-yr-old boy, who accidentally ingested this poison, died within 14 h. Marked insulinopenia, without a reduction in glucagon levels, suggested a specific beta-cytotoxic effect, which was supported after autopsy in three cases by histopathologic evidence of extensive beta cell destruction.
Rx - niacinamide
Dementia pugilistica
Punch Drunk Syndrome
due to recurrent head injury as in Boxer’s. Personality changes, Paranoia, Delusions, Dementia, Parkinsonian signs, Ataxia, Intention tremors
Dementia due to Metal exposure
Lead related:
fatigue, depression, confusion, episodic abdominal pain, acute intermittent porphyria like pain attacks, raised urinary prophyrins, Anemia with basophillic RBC stippling
Rx is with EDTA chelation
Mercury related:
dementia, ataxia, tremulousness, peripheral neuropathies, cerebellar signs, choreoathetosis
Arsenic related:
nausea, weight loss, peripheral neuropathy, Mees lines in nails, pigmentation and scaling of skin, Rx with Dimercaprol
Aluminium related:
progressive encephalopathy as accumulates in brain in ESRD, confusion, aphasia, memory loss, agitation, lethargy, stupor, speech arrest, myoclonic jerks
Seizure types:
- Simple Partial - no LOC,
a general strange feeling that’s hard to describe = a “rising” feeling in your tummy = a feeling that events have happened before (déjà vu)
unusual smells or tastes.
tingling in your arms and legs. - Complex Partial: focal seizures that start in one hemisphere of the brain and are associated with impairment in consciousness = now preferably called “focal impaired awareness seizure” or “focal onset impaired awareness seizure.”
- Partial seizures with secondary generalization are focal seizures that spread to both sides of the brain causing a grand mal convulsion. Except for beginning locally and spreading, partial seizures look the same as the generalized tonic-clonic seizure,
- Absence, petit mal seizures: multiple episodes of absence type, sudden freezing, = 3hz spikes in EEG
- TONIC
- Clonic
- GTCS
- Myoclonic - sudden brief muscle jerks
un classified: -
Infantile spasms, Neonatal seizures
Gullain Barre syndrome
acute inflammatory demyelinating neuropathy, ascending, autoimmune
post Flu or campylobacter GE
ascending symmetrical paralysis
absent DTR ie areflexia
Facial palsy possible
Lower cranial nerve palsies possible
Risk of respiratory failure
autonomic features: Arrhythmias, Hypotension, Diarrhea present
Look for other cause if bladder dysfunction is prominent as brainstem or cervical myelopathy
Normal Pressure Hydrocephalus
Triad of: Ataxia + Dementia + Urinary incontinence
MRI: enlarged lateral ventricles without cortical atrophy and patent aqueduct of sylvius
CSF shows - high opening pressures, otherwise normal. suspected delayed CSF absorption, slow from arachnoid vili into veins
improves with VP shunt
GBS mimics
Botulism (descending paralysis)
Hypokalemic PP
Vasculitis neuropathy
Toxins: Lead, Organophosphates, Thallium, Aresenic,
Diphtheria
Tick paralysis
Shellfish Poisonins
Porphyria
Spinal cord syndromes - quadriparesis
Acute form of CIDP
Lyme polyradiculopathies
Sympathetic Effects
Has survival benefits
(Fight or flight response)
Tachycardia
High Blood pressures
Urinary sphincter tone rise (retention)
Constipations reduced motility
Bronchodiltation
Sweating
Pupillation dilatation
Ejaculation, Orgasm
Parasympathetic Effects
Mostly through vagus
Bradycardia
Hypotension
Urinary incontinence/ voiding
Diarrhea
Bronchoconstriction
Pupillary constriction
Erection
Tearing of eyes
Salivation
OP poisoning activates it
Brachial Plexopathy
Upper Trunk, C5C6 (Birth injury)
- Erb’s Duschene palsy
- jerking/pulling arm down can cause it
Arm: internally rotated + elbow extended + wrist flexion and posteriorly directed (Policeman’s tip)
Weak shoulder abduction+weak external shoulder rotation+weak biceps
Lower Trunk C8T1, (Birth Injury)
Klumpke paralysis- when baby pulled out by arm from birth canal
weak wasted hand, forearm wasting, claw hand, wrist drop
Sensory loss on ulnar side
may have Horner syndrome
Other Causes: Cervical Rib, Thoracic Outlet syn, Tumors, Cx band,
Brachial Cord Lesions
Lateral Cord, C5C6:
= Musculo-cutaneous, Median nerve
= weak flexion & pronation
= sensory loss on radial side of Hand/ forearm, damage due to trauma/ stretching of shoulder
Medial Cord, C8T1:
= Median, Ulnar nerves affected
= weak wasted hand: claw
= sensory loss ulnar side
= Trauma
Posterior Cord, C7:
= Axillary, Radial nerve affected
= Weak deltoid, elbow extensors
= weak wrist extensors, weak finger extensors so flexor strong
sensory loss on outer arm only
- can be with shoulder dislocation/ humerus neck fracture
Leprosy neuritis
cause: mycobacterium leprae
affects cooler part of body
Types: Tuberculous, Lepromatous, & Borderline
Tuberculoid: = single skin patch hypoasthetic, near by nerve thickened
Lepromatous: = more widespread skin & nerves affected with anhydrosis - spares axilla, anterior chest, scalp, groins and affects: Ear pinna, dorsum hand + forearm + back + Legs
deformities develop: claw hand, Finger loss, Foot drop,
inability to close eyelids as weak orbicularis oculi.
ANS parts
Sympathetic ANS:
= T1 to L2
= Superior Cx ganglion
= Middle Cx ganglion
= Inferior stellate Cx ganglion
= Coeliac + other ganglion in abdomen
= Lower abdomen sympathetic ganglion
Para-sympathetics ANS:
= Cranial 3, 7, 9, 10
= Sacral 2, 3
= Ciliary ganglion
= Sphenopalatine ganglion
= Submandibular ganglion
= Otic ganglion
= Vagal ganglion cells (Myocardiac)
= Pelvic ganglion
Lacunar Stroke
Pure-Motor LS:
affects posterior limb of internal capsule, focal weakness or arms/ Legs
Pure Sensory LS:
ventral/ thalamic stroke
Ataxic Hemiparesis:
infarct in ventral pons, int capsule
Syndrome of Dysarthria + Clumsy Hand
Infarct of ventral pons or genu of IC
ACA syndromes
Anterior Cerebral artery
- Contralateral Leg-foot palsy (branch to motor area for leg)
- Contralateral arm palsies: arm area if cortex/ IC fibres
- Cortical sensory loss over toes, foot leg
- Urinary incontinence - sensory motor-paracentral block
- Contralateral frontal release signs
- Abulia, Akinetic mutism (medial FTP)
- Gait apraxia - frontal cortex near Leg motor area
- Dyspraxia-Left limbs, tactile apraxia left limbs (corpus callosum)
Causes of Ptosis+Ophthallmoplegia
- Peripheral neuropathies: GBS, Miller fischer syndrome
- NMJ disorders: MG, Botulism, LEMS, Congenital MG,
- Thyrotoxicosis
- Mitochondrial myopathies: Kearns-Sayre, CPEO
- Congenital myopathy
- Myotonic Dystrophy
- Oculo-Pharyngeal dystrophy
Myopathy clues
Upper face: inability to close eyes forcibly
# Lower face: impaired weak puckering
# Neck Extensors: weak, dropped jaw, cannot raise head well
#Neck Flexors: cannot raised head when lying down without support
#Proximal Arm: unable to raise arms above head
#Knee extensor: Genu recurvatum, back kneeling, unable to walk without hyper-extending knee
#Shortening of achilles tendon - toe walking, cannot walk with heel flat on floor
#Anterior Leg: High steppage Gait, foot drop
#Hip Muscles: Waddling gait, gower’s sign positive (climbs on himself), cannot get up sitting in chair with arms raised.
Causes of Enlarged Nerves
Neurofibromatosis
Leprosy
Amyloidosis
Acromegaly
Refsum disease
CMTD
Hand wasting causes
Anterior Horn cell lesion:
MND/ALS, Syringomyelia, Polio, Cord compression
Brachial plexus: Cervical Rib, Pancoast tumor, Trauma, also will have wasted shoulder and winging of scapula
Peripheral nerve
combined median, ulnar lesions
mononeuritis multiplex
Muscle causea: disuse atrophy in RA
Hypothalamic disorders (ANS)
Horners syndrome
Wernicke-Korsakoff syndrome
Diencephalic syndrome
Neuroleptic malignant syndrome
Serotonin syndrome
Fatal familial insomnia
ADH related: DI-SIADH
Hypo or Hyperthermia
Sexual dysfunction
appetite disorders: Bullimia, anorexia
Disturbances of BP, HR, Gastric function
Acute Disseminated Encephalo-myelitis
ADEM
mimics Multiple sclerosis
Post vaccination, Measles, Rabies
Post infections as Varicella
widespread foci of demyelination
Can follow: Rubella/ Mumps/ Influenza/ Infectious mononucleosis/ Mycoplasma infections
Symptoms: Fever, Headache, Meningism, Lethargy, coma
Seizures are common
Neuro Signs: Hemiparesis, extensor plantars, DTR lost or brisk, Brainstem signs, Lost sensations, Cerebellar (basically depending on area affected)
CSF: proteins high, wbc high, Oligoclonal bands
MRI: extensive gadolinum enhanced plaques
Rx - Glucocorticoids, Plasma exchange, IVIG
CSF in bacterial meningitis
Opening pressure > 18 cm H2O
PCR testing for variety pathogens
WBC count - 10-100, 000/uL
RBC - nil if good tap
CSF/Serum Glucose < 0.4
CSF Proteins - > 0.45 mg/dl
Gram stain - positive > 60%
Culture positve > 80%
Latex agglutination - Strep pneumonie, Meningococcus, H influenza, E coli
Limulus amebocyte lysate assay - detects gram negative endotoxins
Recurrent Falls without LOC
Gait problems: Joint issues, sensory ataxia, cerebellar CVA, Bradykinesia and Rigidity, Confusion
Postural Hypotension: Parkinsons plus, Drugs (BB, Omnic, CCA etc), Autonomic, Addison’s, Anemia, Cancer
Vision Loss, Night blindness
Acute causes - Low BP, Low Hb, low Glucose
LOC with Low BP
Addison’s disease
Autonomic failure
Anemia, Cancer
Medications: beta blockers, ACEi, Diuretics, CCA etc
Cranial Nerve Reflexes
Corneal reflex: V1 branch of Trigeminal to Facial Nerve efferent
Pupillary reflex: 2nd Optic nerve to 3rd Oculomotor as efferent
Jaw Jerk: V3 branch of Trigeminal to V3 branch of Trigeminal efferent
Gag reflex: 9th nerve to 10th vagus
Carotid Sinus Reflex: 9th nerve to 10 th vagus as efferent
Lacrimation: V1 branch of Trigeminal to 7th facial as efferent
Restless Legs syndrome
4 criteria must be fullfilled:
= Akathisia (an inability to remain still)
= Wakeful motor restlessness
= Rest symptoms also
= More to end of day (evening)
Triggers:
Iron, Low B12. Low folate, CKD, Pregnancy, Hypothyroidism, DM, Steroids, Antidepressants, Caffeine
Rx:
correct whatever deficiencies
avoid prolonged sitting as moving improves the restlessness
Avoid trigger as caffeine
LEVO-DOPA therapy
Frontal release Signs
appears in frontal lobe damage which removes inhibition/ control but present normally in babies
1 Grasp reflex - touching palm induces grasping of stimulus
2 Palmo-mental reflex - scratching palm causes mental contraction as chin
3. Suckling, Rooting reflex at touching cheek, lips
4. Glabellar Tap: keeps blinking repeatedly
5. Utilization behavior: Reaching out for and inappropriate use of objects: fiddling with your stetho or bedsheets (not seen with alzeimers disease)
Frontal Lobe Assessments
- Verbal Fluency- words starting with F+A+S = shoulder able to say 15 words per letter in 60 seconds.
- Abstraction - ask abstract meaning of phrases as “Too many cooks spoil the broth” or “ Make hay while the sun shines”
- Response inhibition-Set shifting - inability to shift from one cognitive task to another: (Luria 3 step Test: do Fist-Edge-Palm when told to show)
Frontal Release Signs
Paratonia-Gegenhalten - abnormal tone/ resistance in limb muscles on contralateral side of frontal lobe disease, (inability to relax)
Paratonia - Gegenhalten
Paratonia is the inability to relax muscles during muscle tone assessment. There are two types of paratonia: oppositional and facilitatory. Oppositional paratonia (“gegenhalten”) occurs when subjects involuntarily resist to passive movements,[1] while facilitatory paratonia (“mitgehen”) occurs when subjects involuntary assist passive movements.[2][3][4]
Both types of paratonia have been associated with cognitive impairment or mental disorders, particularly in relation to frontal lobe dysfunction.
Hip Movement Muscles, Roots
Hip Flexion: Iliopsoas, L1L2(femoral)
Hip Extend: Glutei, L4L5S1 (inf gluteal)
Hip Adduct: Adductor longus/brevis/magnus/gracilis/ pectineus, L2L4 (Obturator)
Hip Abduct: Glut max, L4L5 (inf gluteal)
Internal Rotation: Glut minimus (Sup Gluteal)
Ext Rotation: piriformis/ Gemellus superior & inferior, obturator internus & externus, quadratus femoris, gluteus maximus, medius, & minimus, Psoas major & minor, Sartorius.
Knee Movements: Muscles, Roots
Flexion: hamstrings, gracilis, sartorius, gastrocnemius, plantaris, and popliteus.
L5S1 sciatic nerve
(Hamstrings are: Semitendinosus.
Semimembranosus, Biceps femoris)
Extension: Quadriceps - Rectus femoris, Vastus intermedius/lateralis/medialis (L3L4 femoral nerve)
Superior Gluteal Nerve L4L5S1
Hip Abductor
- supplies gluteus max and medius
- supplies Tensor fascia lata
- supplies piriformis
Weakness due to nerve lesion causes weak abduction of same hip and is known as Trendlenberg gait.
The Trendelenburg sign is said to be positive if, when standing on one leg (the ‘stance leg’), the pelvis severely drops on the side opposite to the stance leg (the ‘swing limb’). The muscle weakness is present on the side of the stance leg. Present in paresis of Hip abductors due to superior gluteal nerve damage.
Hyperkinetic Movement Disorders
Athetosis: slow distal writhing involuntary movements of mostly hand
Chorea: Rapid, semipurposeful, graceful, dance like, involuntary, distal or proximal movements
Dystonia: sustained, involuntary muscle contraction, primary/secondary, focal or multiple
Myoclonus: sudden, brief, shock like muscle jerks
TiCS: brief repetitive, muscle contractions, can be motor/ sensory or vocal, simple or complex
Tremor: rhythmic oscillation of body parts due to intermittent muscle contraction - essential/Parkinsons/ Cerebellar
CADASIL - Cerebral Autosomal Dominant Arteriopathy with Subcortical infarcts & LeucoEncephalopathy
- due to mutation in notch-3 gene
- multiple family members affected with recurrent strokes, migraine without HTN,
- progressive dementia in 5th to 7th decade of life
- skin Biopsy: dense bodies in arteriolar media
No treatment except risk factor control and support
Acute Causes of Transverse myelitis
Vascular: infarcts, Herniation of nucleus pulposus
Inflammatory: SLE, Sjogrens, Mixed CTD, Behcet’s disease, Vasculitis
Post Infectious: EBV, HSV, HIV, CMV, Polio
Infections: HSV, EBV, CMV, Rabies, Bacterial, TB, Schistosomiasis, Toxoplasmosis
Sarcoid Myelopathy: Gadolinium enhancement Oligoclonal bands, Uveitis on Slit Lamp, ACE levels high, Chest lesions, Wheeze, lupus pernio
Demyelination cause: MS, Devic’s
Mononeuritis multiplex
Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes less multifocal and more symmetrical. Deep aching pain, worse at night in back/Hip/Legs
common in: DM, SLE, RA, PAN, Churg-Strauss, HIV, Dapsone therapy, Malignancy, Lyme/Leprosy/Sarcoidosis
Typically in DM patients - appears as sudden acute unilateral severe thigh pain followed by anterior thigh muscle weakness & loss of knee DTR
P2 syndromes
Distal PCA occlusion
infarction of medial temporal + occipital lobe. presents as contralteral hemianopia with macular sparing.
Anterior choroidal Artery Syndrome
branch of internal carotid
supplies posterior limb of Internal capsule & white matter.
occlusion of this artery presents as:
= contralateral hemiplegia
= Hemianaesthesia or Hypoesthesia
= Homonymous hemianopia
MCA Occlusion syndromes
Branch of ICA - four main branches, M1, M2, M3, and M4.
complete MCA occlusion:
= contra hemiplegia
= Hemianaesthesia
= Gaze preferences, deviation
= contra homonnymus anopia
= contra Facial UMN palsy
Dominant MCA: Global aphasia
Non Dominant MCA: neglect, constructional apraxia, anosognomia
Pudendal nerve
pudendum means parts to be ashamed of (genitals)
- S2S3S4, travels within pudendal canal, aka Alcock’s canal. SensoryMotor
- Sensory to ext genitalia, perineum anus
- Motor to pelvic muscles(Bulbo-spongiosus, IschioCavernosus, Levator ani) external urethral sphincter, external anal sphincter
- Pudendal block is given through inner wall of vagina. It can be damaged in cycling races, childbirth and leads to fecal incontinence
Onuf’s nucleus
Onuf’s nucleus is a small group of neurons located in the ventral horns of the sacral spinal cord. The motor neurons (MNs) of Onuf’s nucleus innervate striated voluntary muscles of the pelvic floor and are histologically and biochemically comparable to the other somatic spinal MNs. Pudendal nerve carries neurons from Onuf’s nucleus.
Sciatic nerve
Sciatic means pertaining to hip
also known as Ischiadic nerve
L4L5S1S2S3, TRAVELS BEHIND HIP
2 branches: Tibial and Common peroneal.
Supplies in:
Thigh: Hamstrings
Calf: Superficial & deep groups
Sole: Muscles, peronei (anterior compartment & deep)
Sensory: entire sole, dorsum of foot except 1st webspace MT (by deep peroneal) , Lateral side if Leg and calf.
Sural supplies skin lateral edge of foot
Saphenous nerve supplies medial leg and mid dorsum of foot.
Lesion: Gluteal numbness, foot drop, Weak Hamstrings (knee flexion) Weak eversion of foot
DD. -L5S1 radiculopathy
Obturator Nerve
obturator means a device to close body opening - Ob nerve do this by hip adduction.
L2L3L4,
sensory to meal thigh, femoral to front
Motor to Hip adductors
= Adductor magnus
= Adductor brevis
= Adductor longus
= Gracilis
Has 3 branches: Anterior, Posterior & cutaneous
Femoral Nerve
L2L3L4 (Motor 04, sensory 02)
Enters scarpa’s triangle by passing beneath inguinal ligament lateral to femoral artery.
Sensory: Front of thigh, Medial Leg, medial foot
Motor: Iliopsoas, Quadriceps, Sartorius, Pectineus (below inguinal ligament)
Testing Femoral Nerve:
= Hip flexion against resistance
= Knee extension against resistance
= Knee jerk L3L4
= Femoral Stretch Test: Mackiewicz Sign
Lesion: Weak Hip flexion and weak knee extension, numbness along anterior thigh and medial calf
D/D ;L3L4 raduclopathy, DM amyotrophy
l3l4B12 deficiency - SACD-Clinical
similar to Hypocupic Myelopathy due to low calcium levels.
Subacute paraesthesias in Hand, feet
Loss of position & vibration sense
Rhomberg test positive
Progressive spastic-ataxic weakness
Loss of DTR’s + Extensor plantars (LMN + UMN signs mixed)
Optic atrophy in advanced
Symmetric and diffuse damage
Predominant & lateral spino-thalamic and posterior column degeneration
Labs: Macrocytosis, Low B12, High Homocysteine levels, High Methyl Malonic acid levels, + Schilling test (dont using radiolabelled B12)
Rx = 1000 mg of B12 IM then periodic injections and then oral
Hakimov’s Triad
Hakimov triad (gait apraxia, urinary incontinence, dementia) in normotensive hydrocephalus (NPH), although it may be a symptom of some other conditions.
Apraxia of gait is a unique disorder of locomotion characterized by inability in lifting the feet from the floor despite alternating stepping action (frozen gait), and disequilibrium. Responsible site of lesions are in the frontal lobe and/or the basal ganglia.
Gait apraxia is a motor planning deficit and, thus, has a cerebral localization. Patients with gait apraxia have a hard time getting started with walking and may have a “magnetic” or shuffling gait. Gait apraxia is commonly seen in dementia (especially vascular dementia) and in NPH.
Apraxia results in a person’s inability to carry out a familiar purposeful movement, while in ataxia they can carry out the movement with little coordination.
Grading of DTRs
— Absent (minus sign)
+ present only with re-inforcement
+ diminished
++ NORMAL
+++ Hyperactive
++ ++ Clonus
Inverted Reflex: absent reflex at a level and brisk reflexes below that level: indicates cord lesion at level where its absent.
Grading of Muscle Power
05 = normal power
04 = active movement against resistance but not full power
03 = Active movements against gravity but not against resistance
02 = Active movements with gravity eliminated ie side to side only
01 = flicker only
00 = no visible movement
Charcot-Marie-Tooth Disease
Hereditary Sensory Motor Neuropathy
Autosomal Dominant, PPM22 mutation
1. Demyelinating, or 2. Axonal type
gradual progression over decades
clinical signs:
- Pes cavus, foot drop + High steppage gait, inverted champagne bottle appearance as legs atrophic and thighs hypertrophic, variable stocking sensory loss to vibration/proprioseption - rhomberg +
Hand muscle wasting
Palpable lateral popliteal nerve @ fibular head
Diagnosis - Nerve biopsy
Rx - mainly supportive care
UMN Signs
The nerves in the CNS which carry the impulses for movement are known as upper motor neurons (UMN). The primary tract which carries signals for voluntary movement is known as the pyramidal tract. The pyramidal tract divides further into the corticospinal tract and the corticobulbar tract.
Tone - increased, goes onto develop spasticity, clasp-knife spasticity-initial resistance to elbow extension and then suddenly give away,
pyramidal pattern of weakness - UL extensors more weak so they develop flexor spasticity and Leg flexors more weak so they remains in extension when spastic.
DTR’s - brisk, rapid, hyper
Hoffman sign - present, flicking middle finger distal phalanx flexes thumb & index finger
Plantars - extensor
Most common causes of UMN lesions
stroke.
multiple sclerosis (MS)
B12 vitamin deficiency.
Brown-Sequard syndrome (spinal hemiplegia)
trauma to the brain or spinal cord.
tumors.
severe brain infection.
dementia.
Motor Neuron Disorders:
Acute:
Poliomyelitis
Herpes zoster
Coxsackie virus
Chronic:
UMN + LMN both - ALS
UMN mainly - Primary LS
LMN mainly:
- multifocal motor neuropathy with conduction block
- motor neuropathy with cancer/paraproteinemia
- motor predominant + peripheral neuropathy
Other:
MND with other neurodegeneratives
Secondary motor neuron disorders
Multiple system Atrophy (MSA)
Shy Drager syndrome
Multiple system atrophy (MSA) is a rare condition of the nervous system that causes gradual damage to nerve cells in the brain. This affects balance, movement and the autonomic nervous system, which controls several basic functions, such as breathing, digestion and bladder control. can be of 4 types
1. Strianigral degeneration
2. Olivopontocerebellar degeneration
3. MND with Parkinsonism
4. Shy Dragger syndrome
= Postural Hypertension
= Cerebellar ataxia
= Bladder incontinence
= Parkinsonian symptoms-bradykinesia, rigidity, tremor
= Dystonias
= Constipation, Cold hand/Feet, Depression, dementia etc
Toxins/Drugs causing Parkinsonism
Manganese
Carbon monoxide chronic exposure
MPTP ((1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)
Manganese
Rotenone, an insecticide
Cyanide exposure
Alfa methyl dopa
Neuroleptics, Anti emetics
Lithium can cause essential tremor
Reserpine
Fluoxetine
Valproic acid
M-P-T-P
MPTP - is a chemical precursor to the neurotoxin MPP+, which causes permanent symptoms of Parkinson’s disease by destroying dopaminergic neurons in the substantia nigra of the brain)
MPTP was a by-product of the synthesis; it has no narcotic activity. The difference in structure between MPPP and meperidine is slight, and their pharmacological effects are similar. However, esterolysis of MPPP produces a compound that can undergo dehydration to form MPTP.
While MPTP itself has no psychoactive effects, the compound may be accidentally produced during the manufacture of MPPP, a synthetic opioid drug with effects similar to those of morphine and pethidine (meperidine). The Parkinson-inducing effects of MPTP were first discovered following accidental injection as a result of contaminated MPPP.
Manganism, due to Manganese
Manganese overexposure is most frequently associated with manganism, a rare neurological disorder associated with excessive manganese ingestion or inhalation.
Historically, persons employed in the production or processing of manganese alloys have been at risk for developing manganism; however, current health and safety regulations protect workers in developed nations. The disorder was first described in 1837 by British academic John Couper, who studied two patients who were manganese grinders.
Manganism is a biphasic disorder. In its early stages, an intoxicated person may experience depression, mood swings, compulsive behaviors, and psychosis. Early neurological symptoms give way to late-stage manganism, which resembles Parkinson’s disease. Symptoms include weakness, monotone and slowed speech, an expressionless face, tremor, forward-leaning gait, inability to walk backwards without falling, rigidity, and general problems with dexterity, gait and balance. Unlike Parkinson’s disease, manganism is not associated with loss of the sense of smell and patients are typically unresponsive to treatment with L-DOPA.
Symptoms of late-stage manganism become more severe over time even if the source of exposure is removed and brain manganese levels return to normal.
Superior Orbital Fissure Syndrome
Superior Orbital Fissure Syndrome:
SOFS may involve any of the structures coursing through the superior orbital fissure anterior to the orbital apex which include CNIII, CNIV, CNV1, CNVI, and the ophthalmic veins. While superior orbital fissure syndrome may manifest with dysfunction of multiple oculomotor cranial neuropathies, it is dysfunction of the optic nerve function that differentiates orbital apex syndrome from SOFS.
Transient Global amnesia
Short term memory loss: appears confused to due to memory loss of recent ones but long term memories are well preserved. Can tell long term stuff. Asks repeated questions as what is time/place/date etc as forgets.
Lasts around 1-8 hrs and recovered within 24 Hrs which he forgets (day lost)
Usually doesnt recur again.
Association with DM, HTN, Migraine
Suspected Hippocampal ischemia
Rule out - Hypoglycemia, TIA, Stroke, etc
Supportive Criteria for Primary Parkinson’s disease
- unilateral onset (asymmentrical)
- Persistent asymmentry
- Resting tremor (not intentional)
- Progressive symptoms
- Good response to Levo DOPA
- Levo DOPA induced chorea after about 10 yrs of therapy
- Response to L-DOPA > 5 yrs at least
- Clinical course > 10 yrs
Exclusion Criteria for Parkinson’s disease
if present - suspect secondary cause
- Repeated strokes + stepwise progress
- Repeat Head injury as in Boxers
- on AntiPsychotics
- on Dopamine depleting drugs (Olanzapine, Quetiapine, Risperidone)
- Encephalitis or Oculogyric crisis while on no treatment
- More than 1 affected relative
- Sustained remissions, early severe demendia with praxis (loss of skilled functions)
- Negative response to large levo-DOPA
- Strictly unilateral after 3 yrs
- Exposure to known neurotoxin (?mptp)
- Communicating Hydrocephalus +
Plus symptoms: - Supranuclear Gaze palsy, Autonomic disturbance, Cerebellar ataxia, Postural Hypotension
Prevost Sign
conjugate eye and head deviation to side of stroke at rest.
Example - in a right MCA/ACA infarct stroke -disrupted signals to left eye with normal signals to right eye causes as imbalance in neural tone that causes RiGHT EYE to move to right at rest due to no counter pull from left and left eye follows RE due to intact MLF mechanism between 3/6 nerve nucleus.
So both eyes deviates to side of stroke.
Top of Basilar Syndrome
distal end - top of basilar artery OCCLUSION= affects Upper cerebellum, Midbrain, Thalamus, PCA territories.
= Somnolence
= No new memory formation
= Small pupils
= Vertical gaze palsy: cannot look up (3rd nerve partially affected as in midbrain lesion)
PCA infarct
Single PCA infarct:
affects midbrain, Occipital lobes, visual cortex, Posterior temporal lobe and thalamus.
= contralateral hemiplegia
= contra Limb - Motor, Sensory loss
= Left side - reading, naming, color affected
= Right side: Hemineglect to left
Double PCA occlusion:
Cortical blindness or bilateral visual field defects. most common causes are still atherosclerosis, embolism, and small artery disease.
Cerebello-Pontine angle Lesion
Affects- 5, 7, 8 cranial nerves
Common causes - acoustic neuroma, meningioma
Clinical:
- Loss of corneal relfex, keratitis risk
- Facial numbness, weak jaw (5th)
- Facial UMN Palsy, same side
- loss of anterior tongue tase
- Hearing loss, tinnitus
- Vertigo
- Cerebellar dysfunction
- Trigeminal neuropathy symptoms
Cerebral Cavernous Malformation, Familial type
Familial CCMs are inherited through a mutation in one of these genes: KRIT1, also called CCM1. CCM2. PDCD10, also called CCM3.
AVMs are high-flow lesions with rapid blood movement, while CCM lesions are low-flow lesions. The low-flow nature of CCMs makes them angiographically occult and they are only reliably visually detectable using magnetic resonance imaging (MRI).
Cerebral cavernous malformations are removed in a surgery called a craniotomy.
Syncope analysis
True syncope - transient global hypoperfusion: complete transient LOC with rapid spontaneous recovery with no further sequelae or damage.
3 key questions:
1. It is syncopal episode or not? - qualifies above definition.
2. Has the aetiological diagnosis been determined?
3. Is there data suggestive of high risk cardiovascular event or death?
Red flags:
- ECG abnormality
- Heart failure
- Transient lOC or exertion
- Family H/o sudden death < 40 yr old
- Family H/o inherited cardiac diseases
- Age > 65 yrs with no prodrome ie sudden
- a heart murmur
- new or unexplained breathlessness
Get info from Bystanders, paramedic
Eyes usually open during vasovagal/Epilepsy but closed in functional attack
Neuromuscular causes of Ophthalmoplegia
Peripheral neuropathy: GBS, MFS
Neuromuscular Disorders:
= Botulism
= Lambert Eaton myasthenia syn
= Congenital myasthenia
= Myasthenia gravis
Myopathic causes:
= Mitrochondrial myopathy:
Kearns-Sayre syndrome, CPEO, Oculopharyngeal, Myotonic dystrophy (ptosis), congenital myopathy (Nemaline, Myotubular), Hyperthyroidism, Grave’s disease
Stroke Mimics
SDH, EDH
AVM bleed
Normal pressure hydrocephalus
Multiple sclerosis
Mass lesions: Tumors, Abscess
Convulsions: Todd’s paralysis
Metabolic: Hypoglycemia, Hyponatremia
Hemiplegic Migraine
Functional Psychological Hemiparesis
Genetic screening for:
Huntington’s: cytosine, adenine, and guanine (CAG) to repeat
Myotonic dystrophy: mutations in the DMPK gene CCTG repeats
No need to rush, routine type
Send for genetic counselling
Consent is required: confidential
Discuss result implications on patients: - family planning, Career Plans, Insurance plans
Offer better discussion with Genetic counsellors as he can explain better
Family planning options as autosomal dominant:
= IVF and ovum
or embryo selection or
= early prenatal selection by amniocentesis, chorionic villous sampling,
= adoption
Parkinson’s features
3 in Face:
Expressionless face, Blink reflex cannot stop blinking with glabellar tap, Drooling at mouth
3 in Hands:
Pill Rolling tremor + Resting hands in lap + cog wheel rigidity due to tremor + bradykinesia
3 in Actions:
Shuffling festinant gait + micrographia + monotonous speech
3 Plus syndrome:
PSP -upgaze palsy
Postural Hypotension in MSA
Cerebellar signs
Dementia
Tremors:
DoCtoR Wilson HElP
D - drugs
C- Caffeine, Cerebellar
Renal - uremic
Wilson’s disease: copper accumulates
H - Hepatic encephalopathy, hyperthyroidism
E - Essential tremors - improves with alcohol, parkinsons
P - Parkinsonism: pill rolling, Resting
Myotonic Dystrotphy
Autosomal dominan, Mutation in DMPK gene causing CCTG repeats
= myotonic repeats, sad long
= frontal balding
= Bilateral ptosis
= reduced facial expressions
= Testicular atrophy
= Foot drop with High stepping gait
= Hand grip myotonia
= Percussion myotonia
= close eyes tightly, cant open fast
= LIMB WASTING, sensations intact
= Slurred speech
Cataracts early
Pacemakers need Cardiomyopathy, Heart blocks
Glucosuria, DM
Family history
Normal Pressure Hydrocephalus
Triad of Dementia + Urinary incontinence + Ataxic gait
Gait: glued to one place (frozen), unstable posture, tendency to fall back (retropulsion) Upper limb apraxia, Mouth apraxia
Dementia: late sign
Do CT/ MRI
Remove CSF 30-50 ml initially then do cognition check:
= Walking Test
= Reaction time Test
= Memory Test: MMSE
Dysphonia
impaired voice production or phonation due to lesions of vocal cord or larynx or vagal nerve
Step 01: Ask to cough:
= cough normal and speech altered = Local laryngeal lesion
= Bovine cough - Vocal cord palsy
Step 02: Ask to sa long eeeee:
should say it without up/down
if unable to - abnormal
= Monotonous: Parkinson’s (weak bradykinetic muscles
= Laboured: Bulbar weakness
Ortner Phenomenon:
Recurrent laryngeal nerve compression by enlarged Left atrium (LA) causing hoarse voice.
Nystagmus Analysis
Central cause:
- Demyelination, stroke
- may be towards side of lesions
- vertical or rotatory
- Vertigo rare
- Cerebellar signs may be present
- doesnt improve with rest
- slow recovery
Peripheral causes:
- vestibular neuronitis
- fast phase away from lesion
- vertigo common
- tinnitus common
- vomiting common
- improves with rest
- quick recovery due to central adaptation
Dysphasia analysis
confirm dysphasia:
1. Can you tell me ur name and DOB
- Receptive check: Close your eyes - then touch your left ear with right hand - then touch your nose (3 steps)
- Motor check: tell me a little about where you live?
if cannot do both above - GLOBAL type aphasia - Nominal check: Name some common objects - show 3-4 common objects like key, pen etc
- Word finding ability: Name as many animals as you can
- Repetition: Ask to repeat: “ A giraffe is a tall graceful animal”
- Ask to read some thing, write some thing.
Dysphasia Types
Definition: a disorder of language with inability to understand or find words.
Wernicke Aphasia:
Lesion in sensory ie parietal cortex: cannot understand language but speaks fluently, glibly meaningless jargon (sensory, receptive)
Broca’s Aphasia:
(Lesion in motor ie frontal lobe) understands words, commands but unable to express and highly frustrated (Motor, Expressive)
Conductive Aphasia:
intact comprehension and expression but repetition is lost
Trans-cortical Sensory Dysphasia:
same as sensory type but repetition is preserved, lesion in parieto-occipital concept area
Trans-cortical Motor Dysphasia:
same as motor but repetition preserved, incomplete lesion in Broca’s area
Nominal Dysphasia: cannot name objects, angular gyrus lesion.
Causes 3,4, 6 cranials lesions
Brainstem Level:
Vascular strokes
Demyelination MS
Compression by SOL, Tumor
Skull Base:
Infections: Bacterial, Fungal, TB
Carcinomatous meningitis
Neurosarcoid
CNS Lymphoma
Bulbar palsy - 10, 11, 12 mostly
PCA aneurysm - painful 3rd palsy
Temporal Lobe ICH
Petrous temporal tumor in Posterior fossa stretches 6th nerve
Cavernous Sinus: Thrombosis, Sepsis, Carotid aneurysm, Wegner’s
Spinal Cord Lesions - Levels
T2-L1: all lower limb muscles spastic
T8-T12: Abdominal superficial and tickling lost
Hemisection:
CST cut - ipsilateral UMN weakness
DC cut - ipsilateral position, vibration lost
STT - contralateral pain, temp lost
Central Cord Lesion: syrinx
Bilateral loss of pain and temp below level of lesion and other modalities preserved - risk of finger burns, charcot Joints
Dorsal Columns:
affected in tabes dorsalis, long standing syphilis with loss of position and vibration senses, deep pain on pressing achilles tendon lost
Anterior Spinal infarct, (ASA):
Bilateral loss of pain and temperature below lesion. Preserved position and vibration.
Post Spinal Cord trauma problems
High Quadriplegia:, C1-C4
needs respiratory support
completely dependant
Low Quadriplegia, C5-C8
partial dependancy
Paraplegia, below T1
Autonomic dysreflexia, above T6
Headache
Palpitations
Hypertension and tachycardia
Quadriplegic fever:
Recurrent fever with no other cause
Bladder Troubles:
Detrusor spasticity (Rx Oxybutinin)
Urinary dyssynnergia: retention when emptying: Rx with Terazocin
Bladder areflexia
Other issues:
DVT
Pulmonary embolism
Decubitus ulcers
Spasticity Rx: stretching exercises, Baclofen, Tizanidine, Dantrolene, Botulism, Rhizotomy
Lumbo-Sacral Lesions
Upper Lumbo-sacral Lesions:
L2L3L4, femoral, Lat femoral
= weak thigh flexion-adduction
= weak knee extension
= Hip in extension + Knee flexed
= Sensory Loss: Ant thigh, Medial leg
= DTR’s: absent knee jerk
Causes: Diabetic amyotrophy, abdominal Surgery, Lumbo-Sacral plexitis
Lower Lumbo-sacral Lesions
L4L5S1S2, sciatic nerve
weak thigh extension
Weak knee flexion
Weak foot dorsiflexion
Weak Plantar flexion also
Position: Foot drop, Knee extended
Sensory Loss: Posterior thigh, lateral Leg and all of foot
DTR’s : absent ankle jerk
Causes: Plexitis, Surgical; trauma, Cancer, Radiation, infiltrative
LMN Sign
lesion of peripheral nerves beyond anterior horn cells:
Wasting - poor mass
Weakness - reduced tone & power
Hypotonia
Hyporeflexia
Sensory loss
Benign Intracranial Hypertension
(PseudoTumor Cerebri)
Cause of secondary head ache
Usual Case - young woman with obesity in her 30’s.
Migraine like head ache with diplopia, pulsatile tinnitus, loss of peripheral vision (tunneling)
Association with MS, SLE, OC pills, Cerebral sinus thrombosis in pregnancy. (need to exclude those)
Symptoms can include:
Headaches.
Tinnitus (ringing in the ears)
Temporary blindness.
Double vision.
Blind spots.
Neck and shoulder pain.
Peripheral (side) vision loss.
The diagnosis is based on the triad of: (1) papilledema, (2) elevated intracranial pressure with a normal cerebrospinal fluid constituency, and (3) normal central nervous system imaging studies. Treatment involves various maneuvers to lower the intracranial pressure, including medicine and surgery.
Orbital Apex Syndrome
Vision loss from optic neuropathy + ophthalmoplegia
Causes can be:
1. Inflammatory = Sarcoidosis, SLE, Churg-Strauss syndrome, Granulomatosis with polyangiitis , Tolosa Hunt Syndrome, Giant cell arteritis, Orbital inflammatory pseudotumor, thyroid orbitopathy, IgG4 related orbital myositis
- Infectious – invasive fungal sinusitis or post-septal bacterial orbital cellulitis, aggressive bacterial sinusitis without cellulitis causing an OAS.
Fungi: aspergillosis, Mucormycosis
Bacteria: streptococcus, staphylococcus, actinomyces, gram-negative bacilli, anaerobes, mycobacterium tuberculosis
Spirochetes: treponema pallidum
Viruses: Herpes Zoster - Neoplastic = nasopharyngeal carcinoma with extension into the orbit, Primary orbital adenoid cystic carcinoma with extension to the orbital apex
Neural tumors: neurofibroma, meningioma, glioma, ciliary neurinoma, schwannoma
Metastatic lesions: lung, breast, renal cell, melanoma - Hematologic: Burkitt lymphoma, non-Hodgkin lymphoma, leukemia
Perineural invasion of cutaneous malignancy: squamous cell cancer - Iatrogenic/Traumatic –Direct injury typically results from displaced bony fragments leading to anatomic compression of the contiguous nerves and vessels. Indirect injuries result from shearing forces to the anatomic structures of the superior orbital fissure and/or optic nerve from high impact forces.
Iatrogenic: sinonasal surgery, orbital/facial surgery
Traumatic: penetrating, nonpenetrating, orbital apex fracture - Vascular = Carotid cavernous aneurysm, carotid cavernous fistula, cavernous sinus thrombosis
- Other: Mucocele, fibrous dysplasia, neurofibromatosis
Lumbo-Sacral root Pain areas
L3L4 - anterior thigh
L4L5 - Lateral leg, dorsal foot, great toe
L5S1 - posterior calf, heel, little toe
Sciatica - posterior buttocks, thigh
Cauda equina - bilateral thigh-legs
Superficial reflexes
Cremasteric L1L2
Anal wink - S4S5
Abdominals around T10
Plantars - S1 tibial nerve
Enmass reflex: S1 sensory L5 motor - incontinence on flexion of hip and knee
Corneal Reflex - afferent Cranial 5(Trigeminal) and motor cranial 7 (facial)
Peripheral Neuropathy: Common causes
A - Alcohol
B - B12 deficiency, Vegan
C - Cancers, CTDs
D - Drugs, DM
E - Encephalopathy
Hereditary -CMTd
Parkinsonism: Red Flags
suggestive of some thing more than just parkinsons disease
Symmetrical symptoms
Lower Body involvement
Absent/Poor response to L-DOPA
Absence of dyskinesias or fluctuant symptoms after long use of L-DOPA
Early postural instability and falls (suspect Shy Drager syndrome)
Early Autonomic disturbance (Sexual, Bowel, bladder, postural hypotension)
Early pre dominant Upgaze restriction
Early dementia
Early non drug related Hallucinations (suspect Lewy body dementia)
Associated jerks, myoclonus (suspect Multisystem Atrophy)
Sensory neglect: Inattention
Intact cortical vision but when right parietal lobe is damaged patient ignores left side of body which is paralysed and not recognised as existing. if told to raise left side - will not do it.
Needs rehabilitation attempts to stimulate left side movements for example - talk to patient from his left side only
Mirror Agnosia: Looking Glass Syndrome
Patients with sensor neglect due to damage to right parietal lobe when a mirror is placed on their right = showing them there left side body - tries to reach an object through the mirror but not directly if placed on left side as totally neglects left side.
ABCD2 score
prognostic score for TIA patients predicts their risk of stroke in near future
A, Age > 60 = 1 point
B, BP > 140/90 = 1 point
C, clinical weakness, dysarthria without weakness = 1 point
D, Duration of TIA > 60 min = 2 points
and if 10-59 minutes = 1 point
D, Diabetic = 1 point
> 4 points = Do CT/ MRI, Admit, Carotid endarterectomy < 2 weeks
Tremor Types
by Amplitude: Coarse vs Fine
by Frequency: waveforms per second (Hz)
by Anatomy: affected part & symmetry - head, hand, tongue
by voluntary:
- Resting
- Action: Intention, Postural
- Others: Holmes, Dystonic, Primary Orthostatic
Secondary Parkinsonism
Vascular parkinsonism
Drug - MPTP, Manganese
Structural lesions
Huntington’s chorea
Wilson’s disease
Neuroacanthocytosis
Achromotopsia
cortical color blindness due to damage to V4 area, visual in parietal lobe bilaterally, things looks gray
MT area Lesion: middle temporal lobe visual area is involved in sensing movements and in damage here, person might be terrified to cross street but can read books, can recognize faces and see color
Blind Sight
condition with blindness in one visual field due to damage to visual cortex in another cortex. Person cannot see that side but if asked to touch with finger in the field, can do so.
Anosagnosia
denial of left paralysis when right parietal lobe is damaged, sensory cortex
Sensory Neuropathy: Causes
Central Lesions:
= Multiple sclerosis
= Myelopathy
= Stroke, TIA
= Epilepsy
= Tumors
Peripheral Lesions:
= mononeuropathy, Ex median nerve
= Mononeuritis multiplex
= Polyneuropathy
= Radiculopathy
Other causes:
= Migraine with aura
= Panic attacks
= Hypoglycemia
Stroke MIMICs
Seizures with Todd’s palsy
Delirium
Flare up of old stroke
Syncope
Tumor with acute presentation
Psychiatric-Functional-Conversion
Alcohol or Drug related symptom
Hypoglycemia
Binocular Diplopia
Orbital causes: Acute orbitopathy, fractures, cellulitis, tumors
Muscle lesions: MG, Orbital myositis, Kearns Sayre syndrome (CPEO+RP+Young age 20s)
NMJ lesions - MG, Botulism
Nerve Lesions: 3,4, 6 palsies, Orbital apex syndrome, Cavernous sinus syndrome, Multiple Cranial palsies as in meningitis, Wernickes ophthalmoplegia, Miller Fisher Syndrome variant of GBS
Brain Lesions:
Internuclear ophthalmoplegia in MS, Stroke, Tumors etc
Skew deviation: brainstem lesions
Other causes:
Decompensated phoria (heterophoria)
Childhood strabismus with failed compensation
Stroke BP Management
When to lower Blood pressure:
- ICH with BP > 200, MAP > 110
- Hypertensive encephalopathy
- CKD with stroke with High BP
- Stroke with Heart failure
- Aortic dissection
- Pre eclampsia
Use: Labetolol, GTN, Nitroprusside
Secondary Prevention measures for Stroke
ACE inhibitors for BP control
Antiplatelets
Anticoagulants in Afib
Carotid endarterctomy if > 70%
Statins
Life style changes
ICH management
Reverse anticoagulation: Factor 8 concentrates, Vitamin K, Stopd Drug, FFP
Avoid vasidilators
Anti epileptics but not routine
Protect ABC
Surgical approach if:
= Cerebellar bleed > 3 cm
= Young healthy patient with ICH
= Superficial cortical bleed
= Lobar ICH with hydrocephalus
= Lobar ICH with neurodeficits
Cord versus Cauda Equina
Cord compression:
= Spasticity, Hypertonia, Bilateral pyramidal weakness, Brisk reflexes
= Sensory level with loss of all modalities below lesion
= Sphincter disturbance
= Autonomic features
Cauda equina:
= hypotonic flaccid
= absent DTR’s
= Dermatomal loss
= Saddle numbness
= Sensory UB with retention and incontinence
Total Anterior Circulation Stroke
TACS:
Proximal MCA occlusion
Has all 3 of features:
1. contralateral Hemiparesis OR
weakness of 2 of 3 - Face/ arms/ Legs
UMN features
2. Contralateral Homonymous Hemianopia
3. Higher cortical Function abnormality: Dysphagia with left sided TACS, Dyspraxia, Sensory Hemineglect with right MCA stroke
if drowsy but hemiparetic then all 1/2/3 assumed to be present
(ACA is protected by anterior communicating artery which feeds ir from other side MCA)
Lacunar Strokes (LACS)
Lipohyalinolysis of penetrating lenticulo-striate arteries and can affect anterior, posterior circulation areas , basal ganglion or pons
1. Pure motor stroke: anterior limb of internal capsule
2. Pure Sensory stroke: posterior limb of internal capsule
3. Sensory motor stroke
4. Ataxic Hemiparesis: contralateral hemiparesis with ipsilateral cerebellar signs due to small infarcts in basal ganglion or pons.
Partial Anterior Circulation Stroke
due to MCA branch occlusion
Has 2 of 3 features
1. contralateral weakness or Arm/Leg/Hand/Face. Monoparesis may be sole manifestation.
2. Higher cortical dysfunction alone as dysphasia, dyspraxia (inattention or neglect) may be sole manifestation
MCA upper division lesion = hemianopia is less common
MCA lower vision lesions = sensory motor deficits uncommon
Caused of ICH
1 Microaneurysm, fibrinoid necrosis leads to Charcot Bouchard aneurysms in cerebral arteries which might bleed
2. Cerebral amyloid angiopathy: elderly, cortical bleed not deep
3. Tumor with bleed inside
4. AV Malformations:
Lobar bleed from single AV malformations is common in young people leading to SAH mostly, seen with HHT. A large AVM can cause stroke by stealing blood from other terrotories.
5. Saccular aneurysms in young people, also berry aneurysms common in ADPKD, Tuberous sclerosis
6. rare Causes: Vasculitis, Venous thrombosis, Infective endocarditis, Alcohol, Amphetamines, Cocaine, Sympathomimetics, anticoagulations, Thrombolysis
7 Traumatic
Imaging for Stroke - for vessles + Parenchyma and to see reversible ischemia
- Vascular imaging:
CT angio to see occlusion as hyperdense or white vessel, not commonly done for strokes
MIMICS - High Hematocrit, Microcalcification, Hypodence parenchyma might make vessel falsly dense - Imaging acute Parenchymal Infarctions: MR diffusion weighted imaging within 1 hr : CSF looks black in T1 weighted MRI and white in T2 weighted MRI. Infarct looks white in both.
CT will show: Loss of white/gray differentiation, Obscuration of deep nuclei, Loss of insular ribbon - Imaging Reversible Ischemia:
DWI or perfusion MRI
Red flags with Unsteady Gait
Spinal cord signs
Stroke
Guillaine Barre syndrome
Normal pressure Hydrocephalus
Spastic Gait
Pattern: stiff knees with hyperadducted hips close to body, mild plantar flexion at ankles and walks like scissors seen with UMN damage:
= Multiple sclerosis
= Cerebral palsy
= Transverse myelitis
= Motor neuron disease
= Cord compression
= Syringomyelia
= Anterior spinal artery syndrome
= Hereditary spastic paraparesis
= Parasagital meningioma
= Bilateral cerebral infarcts
= Friedrich ataxia
= B1 B12 Deficiency
Spastic Parapresis
walking through mud like gait
bilateral UMN lesions required
UMN Signs: Bilateral Hypertonia, Hyperreflexia, ankle clonus, extensor plantars
- symmetry suggests a complete lesion, Sensory level will localize lesion
- UMN signs in Hand means lesion is above C8 LEVEL
- Examine cranial nerves for evidence of MS or MND
- LMNS signs at level of lesion suggest damage to anterior Horn cells at that level - if LMN signs or widespread, consider MND (hypotonic rather than spastic)
Mixed signs seen in - cord compression, MND, dural AVM at L1L2
Spastic parapresis: Causes
presents as bilateral leg weakness with UMN signs
- parasagital meningioma
- Degenerative disc disease
- Syringomyelia
- Intra dural or Extra dural AVM
- Spinal AVM
- Multiple sclerosis
- Sarcoidosis
- Anterior spinal artery syndrome
- Heredeitary spastic parapresis
- HIV, HTLV-1, B12 DEFICIENCY,
- Cerebral palsy
Evaluation: Acute Neuro-Event
Inquire like this:
1. Before Event:
Doing what, mechanism, past events, any other triggers
2. Suring Event:
Any sugar levels checked?
Any incontinence? stool more serious
Ask as Bed wetting and bed soiling
Ask about any change of face color as went pale(vasovagal) ot went blue(CNS as epilepsy)
3. Recovery: After event:
How did you recover?
Any Sugar given, CPR done, Spontaneous?
Duration of full recovery, short, long?
4. Medication history to take including recreational drugs, OTC, Herbals, Drining
5. Family history to ask as well
Pure Motor Neuropathy
Pure motor Neuropathy: causes
= GBS, CIDP, Multi focal
= Brachial neuropathy
= Diabetic Lumbosacral Radiculoplexus neuropathy
= LMN form of ALS
= Lambert-Eaton myasthenic syn
= Tick bite paralysis
= Poliomyelitis
= Drugs: Dapsone, Lead,
= Acute intermittent porphyria associated
= Diphtheria Motor Neuropathy
Pure Sensory Neuropathy
= Friedrich ataxia
= Idiopathic sensory neuropathy
= Sjogren’s associated
= B12 deficiency neuropathy
= Pyridoxin toxicity
= Cisplatin neuropathy
= Hereditary sensory neuropathy
= Lepromatous leprosy
= DM small fiber neuropathy
= Fabry’s disease
= Vacor associated
Alzeimer’s disease
neurodegenerative disease with progressive cerebral atrophy characterized histopathologically by NEUROFIBRILLARY TANGLES containing TAU protein and BETA AMYLOID plaques in brain matter with apolipoprotein isomer APO-E4 in > 60% above 65 yr old.
= pathology progresses from back to front in brain to memory decline occurs before personality change
= Initial atrophy occurs in Hippocampus, medial temporal lobe
CADASIL: Cerebral Autosomal dominant arteriopathy with Subcortical infarcts and Leucoencephalopathy
CADASIL:
multiple small lacunar infarcts in basal ganglion often with diffuse white matter lesions, mostly caused by:
= Hypertensive microangiopathy
= Low perfusion
= CADASIL or
= amyloid angiopathy of chronic systemic diseases as RA
Creutzfield-Jacob disease (Transmissible spongiform Encephalopathy)
Mutation of Prior prP genes of chromosome 20 turns them into transmissible and change leads to plaque formations.
1. Sporadic type: 85%
2. Inherited, Familial, Auto Dom
3. Acquired CJD: use of cadaveric Growth hormone, consumption of affected peoples brain (Kuru)
4. New variant CJD
Features: Rapid dementia within 2 yrs + myoclonus + Ataxia + Visual disturbances, early death
Charles Bonnet Syndrome
Syndrome of new visual hallucinations in the absence of delirium, dementia or neuropsychiatric disorder
- Mean age of onset 57 yrs
- Hallucination are usually complex, detailed like dwarves wearing silly hats, bright clothing and in occurs broad daylight (no sundowning feature)
- common in macular degeneration.
Encephalopathies causing delirium
MISTIV:
1. Metabolic: Alcohol, drugs, NH3, CO2, O2, Uremia
2. Inflammatory: Lupus, Sarcoid, Behcets, MS
3. Structural: Hydrocephalus
4. Tumors: Primary SOLs, Mets, paraneoplastic( limbic encephalitis)
5. Infections: HSV, HIV, Lyme, CJD
6. Vascular: Venous sinus thrombosis, Vasculitis, SDH, CADASIL
Delirium: VIIT-D-PM
VIIIT-DPM:
Vascular- HTN
Infections: UTI, Pneumonia
Infarctions: STEMI, Strokes
Impactions: U retention, constipation
Traumatic: Head injury edema
Tumors: SOL, Metastasis
Drugs: Alcholol, sedatives
Post ictal
Metabolic, Hyponatremia, uremia, Ammonia, myxedema, Hypercalcemia
AMTS: Abbreviated Mental Test Score
Age
Time of day
Year
Name of place
Recognize 2 people as dr, nurse
Name Ruler of country
Count back from 20
Five minute recall - an address
Neurological causes of Ataxia
Frontal lobe disease
Normal pressure Hydrocephalus
Dementia
Cerebellar ataxia
Spasticity-Hemiparesis, paraplegia
Peripheral sensory Ataxia: Sensory, Visual or Vestibular cause
Peripheral Motor: Myopathy, motor neuropathy
Non-neurological causes Ataxia
Musculo-skeletal: arthritis
Intermittent claudication
Postural hypotension
Fear of falling
Alcohol effect
Medications - sedatives, Antidepressants, Antipsychotics, Phenytoin
Chronic Headache: MOIST-CO
MORE THAN 8 WEEKS headache
M: Medication overuse
O: OSA associated
I: Idiopathic intracranial HTN
S: SOL: Cancers, Prolactinoma, Mets
T: Tension Headache
Co: CO poisoning (wakes up flushed, other roommates affected)
Cluster Headache
primary type headache
also called “Histamine Headache” - ? due to inhibition of nociceptive pathways in hypothalamus
Smoker 80% cases, 50% Auto-dom
May H/o head injury/ Alcoholism
Lasts minutes or Hours, multiple attacks of headache
always unilateral, retro orbital
associated redness eye + tearing
associated runny nose, sweating,
associated ptosis
can be- episodic or chronic
Abortive Rx:
= O2 15 Lit/min for 15 min
= Triptans (contra if IHD, Stroke)
= Ergots
Preventive Rx:
= Calcium channel blockers
= Anticonvulsants
= Galacanezumab/ month
Ischemic stroke: Uncommon causes
Carotid-vertebral dissection
Cerebral vasculitis
Giant cell arteritis
Bacterial endocarditis
Thrombophilia, young pts
Migraine
Patent foramen ovale
Hereditary: Fabry’s disease
Fibro muscular dysplasia, young pt with reno vascular HTN + involves ICA, angio shows beads of strings appearance of artery
Posterior Circulation stroke
Vertebro-basilar, Brainstem
Dizziness, Vertigo, Headache, Diplopia, Loss of vision, Ataxia, Limb Numbness, Limb weakness
Ocular palsies
Oro-pharyngeal dysfunction
Stroke Thrombolysis: Exclusions
exclude from thrombolysis if:
= Age < 18 or > 80
= Time window > 4.5 Hrs
= GTCS on arrival
= GTCS within 6 months
= LP within one week
= Arterial puncture, non compressible
= GIT or GU bleeding < 21 days
= Head injury, stroke, < 3 months
= Past IHC, AVM known, aneurysms
Other possible exclusions:
= Comatose
= Hemiplegia with Fixed head, eye deviation
= Minor symptoms
= Rapidly improving symptoms
= Sx: SAH, even if CT normal
= HTN Bp> 185, DBP > 110
= INR > 1.7, aPTT > 1.2
= Glucose < 2.8 > 22 mmols
= Platelet count < 100k
CT shows ICH or SOL
FAST tool for stroke recognition
Facial weakness
Arm weakness
Speech disturbance
Test all 3 present
Dementia with Lewy bodies
- Fluctuating cognition:
reduced attention and reduced alertness, past H/o recurrent UTI or pneumonias - Recurrent visual hallucination of animals, people
- Sun Downing phenomenon: twilight visits of children, cats as hallucination
- Parkinsonism: bradykinesia, rigidity, festinant gait, monotonus speech, paucity of expression, micrographia
Antipsychotics are contra indicated
Vascular dementias
- Classic multi infarct Vascular dementias - large vessel territories
- Micro-Angiopathic small Vessel disease:
= A. Strategic infarct Dementia: Thalamic, Temporal, Hippocampal
= B. Sub-cortical Arteriosclerotic LeucoEncephalopathy (Binswangers disease)
= C. Multilacunar state
= D. Mixed cortical-Subcortical
= E. Granular cortical Atrophy - Post Ischemic Encephalopathy: CPR
- Cerebral Haemorrhage sequalae
- cerebroVascular+Alzeimer’s: Mixed
Delirium Causes
Age < 65 yrs:
= illicit drug use
= illicit drug withdrawal
= Meningitis
= Encephalitis
= HIV-AIDS
Age > 65 yrs:
= Polypharmacy
= Stroke
= SDH
= UTI, Pneumonia
= Urinary retention
= Fecal impaction
All age groups:
= Electrolyte disturbance
= Hepatic encephalopathy
= Uremic encephalopathy
= Thyroid associated: Hypo/Hyper
= CNS tumors, Mets
= MI, PE associated
= Delirium tremens (alcoholics)
= Wernicke’s encephalopathy
Proptosis: Causes
Grave’s disease
Cavernous sinus thrombosis
Carotid Cavernous fistula
Orbital cellulitis
Retro Orbital tumors: Lymphoma, leukemia, Meningioma, Metastasis
Retro Orbital granuloma: Wegner’s, Histiocytosis-X, Sarcoidosis
Traumatic hematoma
Bamford Stroke Classification
TACS:
Total Anterior Circulation Stroke, MCA-ACA, needs to have all features: Hemiplegia + Homonymous Hemianopia + HIgher cortical dysfunction as Dysphasia, Dyspraxia, Neglect
PACS
Partial Anterior circulation stroke, branch of MCA occluded, 2/3 as above
LACS
Lacunar Anterior circulation strokes
pure motor/ sensory or mixed but small deficit as only hand weakness
Partial Motor Seizures
no LOC if simple partial
Jacksonian March: seizure starts in fingers then marches up along limb
Todd’s paralysis: Localaized palsy after convulsions, recovers completely later
Epilepsia Partialis continua - may continue to have partial seizures for Hours.
Repetitive flexion/extension movements ie clonic
Frequency: 2-3 Hz in EEG
Pure tonic posturing sometimes seen
Hand movements + Facial movements (Twitches & fasciculations)
EEG can be normal if focus is deep seated in brain
Intermittent Myopathic weakness
Evaluation:Check Myoglobinuria
If Present:
clinically normal between attacks
Proximal»_space; distal weakness
Do Forearm Exercise Test: Lactate
*Normal Lactate - CPT deficiency
*Low Lactate - Glycolytic defects
Do muscle Biopsy
————————–
No Myoglobinuria:
Weakness variable, Ophthalmoplegia +, Ptosis +, Bulbar weakness + Limb weakness +
Do Repetitive NCV test
Do Ach receptor antibody testing
- Acquired Myasthenia gravis
- congenital MG
————————
No Myoglobinuria:
Normal between attacks ie no weak
Proximal»_space; distal
Paradoxical MYOTONIA on examine
Normal K = Hyperkalemic periodic paralysis
Low K = Hypokalemic periodic paralysis
Persistent Weakness Evaluation
Look for pattern of weakness
Proximal> Distal = Polymyositis, Dermatomyositis, MD’s
Ptosis, Ophthalmoplegia = Oculopharyngeal MD, Mitochondrial myopathy, Myotubular myopathy
Facio-Scapulo-Humural MD, winging
Facial+Distal quadriceps weakness +Hand Grip myotonia = Myotonic MD
Proximal weakness + Hand grip myotonia + Quadriceps weakness = Inclusion body myopathy (IBM)
Distal weakness only = distal myopathy
Droopy head - MG, PM, ALS
Myopathic EMG confirms muscle disease, Repetitive NCV for MG and other tests as CK levels, DNA testing, Muscle biopsy to do
Muscle Tone assessment
Normal, Hypo or Hypertonic
Rigidity & Spasticity- Hypertonia
Clasp-Knife Spasticity:
if resistance increases when you are moving a joint then suddenly gives away. Example- Heel goes off bed when suddenly flexed passively OR passive flexion at elbow initially resisted then gives way.
Lead Pipe Rigidity:
tone is rigid throughout movement seen in Parkinson’s disease
Cog Wheel Rigidity:
jerky irregular breaking movements like a cog wheel, best assessed by slow movements, seen in Parkinsons
Paratonia-Gegenhalten: refers to resistance due to frontal lobe damage, involuntary resistance to passive limb movements, increases with degree of dementia
AEIOU-THRIPS: Delirium causes
Definitiion: acute confusional status of sudden onset with diurnal fluctuation, inattention, reduces focus, disorganized thinking, cloudy consciousness. Hypoactive or seen restless plucking at bed sheets.
A- Alcohol
E- Epilepsy
I- Insulin ie Hypo/Hyperglycemia
O- Oxygen ie hypoxia
U- Uremia
T- Trauma SAH, SDH, EDH
H- Heart issues: CCF, Arrhythmias
R- Retention of Urine
I- Infections, UTI, Pneumonias
P- Poisons: Oversodes, Sedatives
S- SLE cerebral vasculitis, Autoimmun
Dementia
Impairment of memory to new learnings with any one of = Aphasia, Agnosia, apraxia or Executive function
Irreversible Causes of Dementia:
= Alzeimer’s disease
= Vascular dementia
= Fronto-temporal Dementia
= Dementia with Lewy bodies
Potentially Reversible Causes:
VVIT-A-Hypothyroidism
V- Vasculitis
V - Vitamin B12 deficiency
I - Infections: HIV, Syphilis
T - Tumors, frontal lobe SOL
A - Alcohol excess
H - Hypothyroidism
H - Hydrocephalus (+_ depression)
Genetics of MND
5-10 % cases hereditary/ Genetical
1. UMN + LMN MND:
AutoDom - Superoxide dismutase mutation
AutoRecessive - Alsin mutation
Mitochondrial: Cytochrome oxidase
- LMN type MND:
Spinal muscular atrophies
X Linked Spinobulbar muscular atrophy
GM2 Gangliosidosis: Sandhoff disease, AB variant, Adult Tay-Sach’s disease - UMN type MND:
Selected Familial Spastic paraplegia
AutoDom - Spastin
AutoRec - Spartin
X Linked
Adrenomyeloneuropathy - ALS Plus Syndromes:
ALS with Fronto-temporal dementia
ALS with Parkinsonism
ALS with Tau protein
Seizures: Other Differentials
Syncopes: Vasovagal, Cardiac, situations
Psychogenic Seizures, Hyperventilation, Panic attacks
Metabolic: Hypoglycemia, Hyponatremia, Hypernatremia, Hypoxia
Drugs: Hallucinogens, Ethanol withdrawal
Migraine: confusional, Basilar
TIA of basilar artery
Sleep Disorders: Narcolepsy, Cataplexy, Benign sleep myoclonus
Movement Disorders: Tics, Non Epileptic myoclonus, Choreoathetosis
Pediatric Group: Breath Holding spells, BPPV, Migraine with recurrent tummy pain, Vertigo, Apneic episodes, Night tremors, Sleep walking
Medial Temporal Lobe Epilepsy
Febrile Seizures - familial - Early onset - Remit and reappear, Aura common, behavior arrest, stare, complex automatisms, Unilateral posturing, Post ictal confusion, Memory loss of event
# Work up:
EEG-Anterior temporal spikes (Uni/Bi)
PET scan - hypometabolism in inter ictal phase
SPECT - hypoperfusion interictal
WADA testing: intracranial amo-barbital-material specific memory deficits (tests each side of brain differently for memory issues)
MRI scan: small hippocampus and temporal lobe, enlarged temporal horn
Pathology: Highly selective loss of specific cells in hippocampus
Convulsions in Neonates
in Neonates:
= Perinatal Hypoxia
= Intracranial bleeding
= Trauma to head
= Acute CNS infections
= Metabolic: Hypocalcemia, Hypoglycemia, Hyponatremia, Hypomagnesemia, pyridoxine deficiency
Drug withdrawals - cocaine, Alcohol (mother abusing them)
Convulsions in Children
Febrile convulsions
Genetic, Hereditary
CNS infections
Head injury
Developmental disorders
Idiopathic
Drugs induced
Convulsions in Young and adults
Tumors
Alcohol withdrawal
CNS infections
Drugs induced-Amphetamines
Drug overdoses, Poisoning
Hypoxia associated
Stroke sequelae
Metabolic: Na, Glucose, Ca, Mg, TSH
Idiopathic
Movement Disorders
Akinetic Rigid (Parkinsonism)
Tremors
Dystonia
Chorea
Myoclonus
Tics
Drugs induced movements disorders
Dyskinesia: Drug induced choreas, dystonias, (Metoclopramide)
Acute Dystonia: Haloperidol, Metoclopramide, Prochlorperazine, Antipsychotics
Tardive Dyskinesia: continuous involuntary movements, due to anti-psychotics (tardive means late occurring, months or yrs after Rx)
Epilepsy associated Genes
- Classic Lissencephaly with Severe mental retardation + Seizures, Xq21-24
- ADNFLE - AutoDom nocturnal frontal lobe epilepsy, childhood onset, 20q13.2
- BFNC - Benign familial nocturnal convulsions, AutoDom, KCN62, 20q 13.3, starts first week of life
- GEFS - Generalized epilepsy with febrile seizures, 19q12.1, SCN1B, AutoDom, febrile convulsions at 1 yr
- ADPEAF - AutoDom partial epilepsy with auditory features, 10-25 yrs, Lateral temporal lobe involves, LG11, 10q24, AUDITORY APHASIA
- PME - Progressive myoclonus disease, CSTB, 21q22.3, AutoREC
- Lafora disease: myoclonic epilepsy, EPM2A, 6q24 early dementia, myoclonus
Advice to Epileptics
Stop: = driving, swimming, recreational drug use,
Reduce: = Alcohol < 14 U/Wk, late night awake,
OC pill might be less effective so take barrier precautions as well
If female, planning pregnancy, need to be aware about teratogenic effects and need to plan conception
Gait analysis
Reduced arm swing: Corticospinal tracts, Parkinsons
Stooped posture, Short, shuffling = Parkinson’s
Broad based, feet apart, unstable gait = Ataxia - sensory/ cerebellar
Scissoring gait, crossed steps = Spastic
High stepped, slapping - foot drops due to neuropathy, Posterior column disease
Stuck in, Glued to spot - apraxia with frontal lobe disease
Genetic Testing
done to diagnose disease or delay or prevent complications. indicated early if symptomatic, bad family history, high risk jobs as pilot, military
HOCM = AutoDom, as soon as possible - then ICD insertion
= HOCM ECHO positive: repeat ECHO - if repeat also positive - do genetic studies
= HOCM ECHO negative - repeat later - if still negative - genetic studies if family history
= HOCM ECHO in children: do at 10 yrs then every 3 yrsly upto puberty - then yearly until 18 yr old - if negative by then, every 5 yearly
ATAT deficiency: AutoRec, as soon as possible, to prevent or minimize complications of COPD, cirrhosis
ADPKD: AutoDom, reoutine after age 20, only USG enough for watch and screening
Huntington disease: AutoDom, routine as early diagnosis might be depressive
Myotonic dystrophy: AutoDom, routine as clinical diagnosis enough and early diagnosis offers no benefit
Headache Differential:
Primary Headaches - Migraine, Tension, Cluster Headache,
Secondary Headaches: Meningitis, SAH, Cerebral Sinus thrombosis, Anti-phospho-lipid syndrome, Posterior Circulation Ischaemia, Analgesic Overuse Headache, Sinusitis, Otitis media, Trigeminal neuralgia, SOL
Combined Oral contraceptive pills
Headache Red Flags
= Headache with head injury – a fall or accident
= Sudden headache & extremely painful
Severe Headache with:
= sudden problems speaking or remembering things
= loss of vision
= feel drowsy or confused
= very high temperature and symptoms of meningitis
= the white part of the eye is red
a headache with vision problems or difficulty speaking, swallowing, balancing or walking
a headache with drowsiness or a persistent lack of energy
a headache that starts within 5 days of a head injury
Tension Headache
Common symptoms of tension headaches include:
= pain on both sides of your head, face or neck
= feeling like something is pressing on your head or being tightened around it
= the affected area may feel tender and your head may hurt more when touched
= You should be able to continue doing daily activities without making the headache worse.
Tension headaches last at least 30 minutes but they can last much longer, sometimes for several days.
Causes of tension headaches
= stress
= sleep problems
= caffeine
= Overuse or rebound headaches.
Tension headaches are not a sign of an underlying condition.
CNS Radiation Toxicity
Early delayed Radiation Toxicity: < 4 months - somnolence in children, Lhermitte phenomenon after cord radiation
Rx is with Glucocorticoids
Late Delayed Radiation Toxicity: > 4 months after: Radiation necrosis (low glucose uptake in PET-SPECT,
contrast enhancing mass noted
MRI Spectroscopy required-high lactate, low choline
develops - dementia, hypo-pituitarism, new cancer like lesions but low glucose uptake
GBS Subtypes
AIDP: adults 90%, Anti GM1 antibodies+ first attacks schwann cell surface, widespread myeline loss
AMAN - Acute Motor Axonal neuropathy: in children, Anti GP1 antibodies, axonal damage starting at nodes or Ranvier (no sensory)
AMSAN - Acute Motor-Sensory neuropathy, Adults, axonal damage, sensory loss also seen
Miller fischer type: uncommon, adult & children, Ataxia + Ophthalmoplegia + Areflexia. Anti GQ1 antibodies +, demyelination present
Rx - IVIG, Plasma pheresis
GBS diagnostic Criteria
Must present:
= progressive weakness or 2/ more limbs
= Areflexia
= Less than 4 weeks duration of start
Exclude: Vasculitis/ Toxic/ SLE/ Churd strauss
Supportive features:
- relatively symmetric
- Mild sensory mostly motor
- facial, lower cranials involved
- no fever
- CSF: Acellular with High proteins
- Demyelination in NCV studie
may have Back pain, Hip Pain
Tension Headache
Common symptoms of tension headaches include:
= pain on both sides of your head, face or neck
= feeling like something is pressing on your head or being tightened around it
= the affected area may feel tender and your head may hurt more when touched
= You should be able to continue doing daily activities without making the headache worse.
Tension headaches last at least 30 minutes but they can last much longer, sometimes for several days.
Causes of tension headaches
= stress
= sleep problems
= caffeine
= Overuse or rebound headaches.
Tension headaches are not a sign of an underlying condition.
Cluster Headache
quickly and without warning, pain very severe, sharp, burning or piercing sensation on one side of the head. It’s often felt around the eye, temple and sometimes face. It tends to affect the same side for each attack. People often feel restless and agitated during an attack because the pain is so intense, and they may react by rocking, pacing or banging their head against the wall. May Have:
= a red and watering eye
= drooping and swelling of 1 eyelid
= a smaller pupil in 1 eye
= a sweaty face
= a blocked or runny nostril
The attacks generally last between 15 minutes and 3 hours, and typically occur between 1 and 8 times a day.
= Usually same time every day
= Cause unclear, link to Hypothalamus
= More in smokers
= Genetic link suggested
= Aggrevated by Alcohol, Strong Smells, Petrol
Pattern of attacks= usually happen every day, in bouts lasting several weeks or months at a time (typically 4 to 12 weeks), before they subside.
A symptom-free period (remission) will often follow, which sometimes lasts months or years before the headaches start again.
They’ll often get cluster headaches every year for many years and they may be lifelong. They tend to happen at similar times of the year, commonly in the spring and autumn.
Nerve Conduction Velocity (NCV)
involves electrical stimulation of a peripheral nerve recording latency and amplitude along it. From latency, conduction velocity can be calculated.
Normal Conduction Velocity is:
= 50- 60 m/Sec in arms
= 40-50 m/Sec in Legs
Amplitude is small & large Nerve size #Demyelination: Velocity is slow, amplitude is preserved.
#Axonal neuropathy: amplitude is low, velocity is normal. Reduction of amplitude of recorded responses generally indicates a loss of axons.
Acute neuropathy - upto 4 weeks
Chronic Neuropathy: more than 8 wks
Neuropraxia - focal of segmental demyelination with preservation of the axon, recovers in 2/12 weeks
Axonotmesis: axon is divided but the epineurium remains intact and regrowth occurs at 1 mm/day from injury site
Autonomic Chemical Tests
- Serum Nor-epinephrine:
Supine NE- reduced in postganglionic lesions = Standing NE at 5 minutes - fails to rise in pre-ganglionic lesions - Tyramine administration - causes NE to be release at post-ganglionic ends and is reduced when lesion is post-ganglionic.
- Phenyl Ephrine (an alfa agonist) administration - shows excessive NE response even at low levels
- Trimethaphan (a nicotinic antagonist) causes ganglionic blockade and can induce hypotension if lesion is pre-ganglionic. production of controlled hypotension during surgery to reduce bleeding into the surgical field and also for rapid reduction of blood pressure in the treatment of hypertensive emergencies, especially in patients with acute dissecting aneurysm, and in the emergency treatment of pulmonary edema in patients with pulmonary hypertension associated with systemic hypertension.
Dementia, Reversible Causes
Thiamine deficiency, early
Hypothyroidism
Vitamin B12 deficiency
Normal pressure Hydrocephalus
Subdural Haematoma
Chronic infections
Brain tumors
Drug intoxications
Periodic Paralysis (non dystrophic myotonia)
- Ca channel related: HypoK-PP
The most common familial form, type 1 HypoKPP, has a mutation in the dihydropyridine-sensitive, skeletal muscle calcium channel gene, CACNA1S. Type 1 Hypokalemic periodic paralysis - Autosomal dominant, starts in adolescence, no myotonia, attacks daily or yrly, K low during weakness, doesnt change with K infusion. - K channel - Anderson Syndrome:
AutoDom, Early childhood onset, No myotonia, episodic weakness, Daily to yrly attacks last 2-24 Hrs, No change with K loading or muscle cooling. - Na channel related
Hyperkalemic periodic paralysis
Paramyotonia congenita
Hypokalemic Periodic Paralysis,
Acute Treatment
Acute Treatment:
The goal is to normalize the serum potassium level by administering oral potassium chloride. Oral potassium chloride is administered in incremental dose, starting initially with 0.5 to 1 mEq/kg (i.e., 60 to 120 mEq of potassium for a 60 kg individual) is reasonable.
If they do not respond to the initial dose, then 30% of the initial dose (i.e., 0.3 mEq/kg) is repeated every 30 min.
If the patient requires the addition of more than 100 mEq of oral potassium, then close monitoring of serum potassium is needed, and the total dose of oral potassium should not be more than 200 mEq within the 24 hours of starting of the treatment.
do ECG monitoring, and muscle strength should be examined periodically. Serum potassium level should be monitored for 24 hours after treatment as the post-treatment rise in serum potassium level can have an adverse effect on patients.
IV potassium is not preferred initially and is reserved for arrhythmias due to hypokalemia or if the patient has swallowing difficulties or respiratory muscle paralysis.
IV potassium is preferentially administered with the mannitol, not with dextrose or saline as both carbohydrate and salt can itself trigger the muscle paralysis and thus may worsen the weakness.
IV potassium therapy requires inpatient, continuous ECG monitoring. 40 mEq/L in 5% of mannitol solution of IV potassium is infused at a rate not more than 20 mEq/hour, not exceeding 200 mEq in 24 hours.
Individuals having a milder form of attacks can also benefit from low-level exercise.
Periodic Paralysis, prevention of attack
Pharmacologic interventions include medications like chronic potassium supplementation, carbonic anhydrase inhibitors (CAIs), potassium-sparing diuretics that are used when lifestyle modifications become insufficient in reducing attack rates. The favored approach is to add one of the diuretics with the chronic potassium supplementation. The initial choice of diuretics is carbonic anhydrase inhibitor acetazolamide.
D/d for Primary HypoK Periodic paralysis
Hyperkalemic or normokalemic periodic paralysis
Thyrotoxic periodic paralysis
Andersen-Tawil syndrome
Secondary hypokalemia
Myasthenia gravis
Paramyotonia congenita
Autonomic disorders due to spinal level causes
Traumatic quadriplegia
Syringomyelia
SACD
Multiple sclerosis
Tetanus
Amyotrophic Lateral sclerosis, ALS
Stiff Man syndrome (alternating rigidity and spasm in their muscles. Muscles can be so rigid that they feel like a board. And the muscle spasms, which are extremely painful and can be triggered by stress or other external factors, can create enough force to break bones.F)
Headaches Differentials
Exclude if acute: SAH, meningitis, Carotid-Vertebral dissection, Cavernous sinus thrombosis, Pituitary apoplexy
Primary Headaches:
1. Migraine with or without aura
2. Tension Headache
3. Trigeminal autonomic cephalgia-Cluster Headchae
4. Medical Oversuse Headache
5. Exertional Headache
Secondary Headache:
Infections - meningitis, abscess
Neoplastic: Brain or metastatic
Vascular: SAH, GCA, Venous thrombosis, Extracranial dissection
Traumatic: Head or neck injury
Referred pain - Otitis, Glaucoma, Sinusitis
Headache Features
Tension Headache: usually end of day, band around head, bilateral and across forehead, improves with rest
Migraine Headche: unilateral, throbbing, with or without aura, frontoparietal, can have transient hemiplegia, prefers dark quiet room
Cluster Headache: always unilateral, retro orbital, eye watering, redness, many clusters in a day
Temporal Arteritis: temporal headache with tender scalp, eye pain, risk of blindness, red desaturation, RAPD, pale optic disc, needs urgent steroids
SAH, meningitis - occipital, stiff neck, photophobia, sudden thunderclap in SAH, fever, rash, contact history
Drugs induced
Optic neuropathies
Demyelination as in MS
Vascular - AION (GCA) , Non arteritic AION in vasculitis, anemia, Hypotension, CTDs
Compressive - meningioma, Glioma, metastasis
Nutritional: Thiamine, B12, Folate def
Drugs/ Toxins: Ethambutol, Amiodarone, INH, Lead, CO
Orbital trauma, Syphilis, Lyme
Hereditary: Congenital Lebers Hereditary Optic neuropathy
Headache analysis
Onset/Site/ Duration/ Progression/ Aggravating factors/ Relieved by/ Associated features
Triggers: Exercise, certain foods as chocolates, Cheese, pre-menstruation, Bright lights, Sounds, Lack of sleep,
Analgesic overuse headache > 12 days per month use of painkillers for headache.
Risk factors for serious causes: - Past malignancy, Arthritis (? SLE), HIV risk factors cause odd CNS infections, Smoking, Alcohol, Illicit drugs, Contact with possible Meningitis,
Local Pain Sources: TM joint pain, Temporal arteritis, Herpes rash, C SPINE tenderness, Greater occipital nerve tenderness, eye problems, ear, mastoid
Absent Ankle DTR + Extensor Plantar conditions
combination of UMN (extensor plantar) and LMN (absent ankle reflex) seen in some conditions as:
1. Cervical myelopathy with DM sensory neuropathy
2. MND with lesion at conus medullaris
3. SACD with B12 deficiency (also can cause pure sensory neuropathy, dementia)
4. Tabes Dorsalis due to tertiary syphilis after about 20 yrs of untreated primary syphilis - cellular reaction in dorsal root ganglion causes retrograde sensory tract loss, initial lancinating pain attacks then loss
5. Friedrich ataxia: GAA repeats, AutoRecessive, degeneration of spinocerebellar, Doral column, corticospinal tracts causing mixed signs. Progressive ataxia below 25 yr old, EMG+NCV suggestive of axonal neuropathy,
SACD vs Tabes Dorsalis vs Friedrich ataxia
Dorsal columns degeneration in all 3
Rhomberg test positive in all 3
Spinothalamic tracts spared in all 3
Absent ankles reflex(LMN) in all 3
Extensor plantar reflex (UMN sign) in all 3
SACD: Optic atrophy with centro-caecal scotoma, Dementia, Pernicious anemia
Tabes dorsalis: Charcot Joints, Argyl-Robertson pupils, Autonomic degeneration, Neurgogenic bladder, constipation
Friedrich Ataxia: Pes cavus + Kyphoscoliosis as long standing neuropathy, Cerebellar signs, DM, Cardiomyopathy
Chronic Demyelinating Polyneuropathy
Genetic/ Hereditary:
= CMT type 1,4, X linked
= Hereditary Liability to pressure palsies
= Metachromatic Leucodystrophy: Lipid build up
= Globoid cell Leukodystrophy: Krabbe’s disease
= Refsum disease: Phytanic acid build up (Peroxisome deficiency)
Acquired CDP:
= CIDP: Autoimmune, High CSF proteins
= Multifocal Motor Polyneuropathy
= Paraproteinemic demyelinating polyneuropathy
= Monoclonal Gammopathy of Uncertain Significance, HIgh Globulins
= Osteosclerotic myeloma
Progression of Distal Symmetrical Polyneuropathy
Predominant Axonal DSP: DM, Vasculitis, Toxins
Predominant Motor DSP: Charcot-Marie-Tooth
Initial distal symmetrical sensory symptoms
Ascending sensory symptoms
Weakness of toe, foot dorsiflexion (cant stand on toe or heels)
Loss of ankle reflexes
Relatively preserved plantar flexion (cant stand on toes ie weak dorsal flexion but good plantar flexion)
Shin & Finger tip paraesthesia
Gait Disorder: Lost proprioception, weak extensors
Mid thigh, upper forearm & Lower abdomen Hypoesthesia
Advanced: Stock & Glove sensory loss, distal wasting and weakness, Absent reflexes
Syringomyelia
Syrinx is an empty cavity which starts in center of brainstem or upper cord and expands which damages neural tracts with effects
Dissociated sensory loss ie loss of pain, temperature but preserved vibration and position sense and differentiates it from DM/ Tabes dorsalis.
Clinical:
= Horner’s syndrome
= Loss of hot/cold discrimination & pain in cape like distribution & both UL (C2)
= T1 root signs: Wasting of small hand muscles
= Loss of Upper Limb DTRs
= Flaccid Tone in Upper Limbs
= Spared dorsal columns (no Rhomberg)
= Late: affects CST: Spastic paraparesis
= Charcot Joints (neuropathic)
= Cervical Rib might be present
= Kyphoscoliosis
La Main Succulemente: Cold, swollen, dystrophic hands that may occur in syringomyelia
Spastic Paraparesis
above C4 causes tetraparesis
Clinical: Spastic limb tone with weakness, clonus, hyperreflexia, extensor plantars, root signs might be present (UMN lesion)
in Brown-Sequard Syndrome: contralateral pain and temperature loss, ipsilateral position vibration, spastic paraparesis
Compressive Causes of SP:
= Cervical spondylosis
= Disc prolapse
= Tumors, Trauma to cord
= Meningioma, Astrocytoma, Neurofibromas
Non Compressive Causes of SP:
= Multiple sclerosis
= Viral infections
= Autoimmune as SLE
= Infarction, Bleed in cord
= Friedrich ataxia (degenration)
= Spino-Muscular atrophy
= Hereditary Spastic Paraparesis
= West Nile virus, HIV, Syphilis, Lyme
= SACD, Vitamin E deficiency
= Lathyrism, Konzo disease
in Cortical Lesions with Quadriplegia: Bilateral cortical
Konzo disease
Konzo is an epidemic paralytic disease occurring among hunger-stricken rural populations in Africa where a diet dominated by insufficiently processed cassava results in simultaneous malnutrition and high dietary cyanide intake.
Myelopathy Work up and Treatment
Work up:
Early MRI spine: High signal on T2
CSF analysis: including Oligoclonal bands
Treatment:
- Surgical decompression
- IV Acyclovir 10 mg /Kg/TID if HSV myelitis??
- Pulses Methyl Pred 0.5-1 GM OD x 3 days
- Hydroxycobalaime 1000 mcg OD x 5 days then every month once
- HAART therapy for HIV
Pain control
Intensive Physiotherapy, Air Bed
Small Hand Muscle Testing
Abductor Pollicis brevis (APB): thumb Abduction, test by Palm, thumb to ceiling, dont let me push down your thumb to palm
First Dorsal Interossei: index finger: Test as palm up and push index out against resistance
Abductor digiti minimi, little finger abduction
tests by pushing out little finger against resistance
APB weak + 1st DI weak = T1 radiculopathy
APB weak + 1st DI spared = Median nerve lesion
APB intact + 1st DI weak = Ulnar nerve lesion
Causes of C8/T1 root lesions
= cervical spondylosis
= Syringomyelia
= MND
= Pancost tumor
= Cervical Rib
Weak Hand Muscle Causes
C8T1 root lesions
Polyneuropathies
Disuse atrophy as in Long Rheumatism
Ulnar + medial lesions
Obturator nerve Lesions
due to plevic tumors or surgery
Weak Hip adduction & sensory loss over medial thigh
Same can be seen with Lumbo-sacral plexopathy
Lateral Spinal Lesion at L2L3L4
Neurofibroma at L2L3L4
Anterior thigh pain
Quadriceps wasting, weak knee extension
ankle inversion (L4) weakness
Absent ankle DTR or Brisk ankle + extensor plantar if terminal cord compressed
Sphincter dysfunction, incontinence
Midline Lesions in Cauda Region
Intrinsic Lesions: Dermoid tumor, Lipoma, Ependymoma
Extrinsic Lesions: Disc protrusion, Sacral tumors, Bone metastasis
Root damaged in to out, S5-S4-S3
Ankle reflex Lost (LMN sign) S1L5
Genital Pain, rectal pain without local pathology
Perianal numbness
Saddle numbness, area sits on horse saddle
Anal wink reflex weak or lost
Rectal tone weak or lost
Acute Flaccid Paralysis Differentials
Brainstem Causes: Bleed, Stroke, Encephalitis
Myelopathic: SOL, Acute transverse myelitis
Anterior Horn Cell: Poliomyelitis, Other virals
NMJ disease: MG, Toxins
Myopathies: Hypokalemia, HypoPhosphatemia,
Inflammatory myopathies, Acute rhabdomyolysis, Periodic paralysis
Peripheral Neuropathies: GBS, Diphtheria, Botulism, Heavy metals, Toxins, Drug induced, Vasculitis, Acute intermittent porphyria, Critical illness, Lymphadenopathies, Post Rabies vaccination AFP
Neuropraxia
Focal or segmental demyelination with axon preservation and recovery occurs in 2-12 weeks
Axonotmesis: Axon divided but epineurium intact and regrowth occurs at 1 mm/day
Neurotmesis: complete division of neuron and no recovery or regrowth possible
Allodynia:
Pain after a non painful stimulus ie pain after light touch
Paraesthesia
Abnormal sensation evoked or spontaneously and not necessarily unpleasant
Dysesthesia: Unpleasatn paraesthesia
Hyperesthesia: increased sensitivity to a stimulus
Autonomic Symptoms
Postural Hypotension
Paroxysmal Hypertension
Tachycardia or bradycardia episodes
Atonic bladder
Constipation
Erectile dysfunction
Dry eyes and dry mouth
Anhidrosis ie loss of sweating
Hyperhidrosis rarely
Diarrhea rarely
Meralgia paraesthetica
Pain and tingling at lateral mid thigh due to damage to lateral cutaneous nerve of thigh damaged at inguinal ligament
Common causes:
= Obesity
= Pregnancy
= Surgery
Volkman’s Ischemic contracture
due to brachial artery tear in supra condylar fracture of humerus
Foot Drop Causes
Common peroneal nerve palsy
Sciatic nerve palsy
L4L5S1 root lesions
Polyneuropathy as CMT disease
UMN lesions involving cord and cortex
Diabetic amyotrophy
pain, weakness and wasting of quadriceps and thigh due to lumbo-sacral plexus micro vasculitis
Shoulder Movement Muscles
Flexion: forward movement: Pectoralis major, Deltoid (anterior 1/3), coracobrachialis
Extension: Backward movement: Deltoid (posterior 1/3rd), Lattisimus dorsi, Pectoralis major, Teres major if shoulder already outstretched to side
Ab-duction: Supra spinatus for first 15 degrees then deltoid
Ad-duction: Pectoralis major, Deltoid middle 1/3rd, Subscapularis
Medial Rotation: Pectoralis major, Deltoid middle rd, Lattisimus dorsi, Teres major, Subscapularis
Lateral Rotation: Teres minor, Infraspinatus
Anterior Cord Syndrome
Loss of power and pain below injury but preserved touch & Proprioception. Injury from front of cord.
Spinal Cord Tracts
Posterior Cord Syndrome
Loss of sensation but power preserved power below lesions.
Central Cord Syndrome
usual mechanism is in elderly people who fall on their chin and cause extension injuries. Incomplete tetraparesis with upper limb paresis more than lower limb. Lower limb power may be normal. Sensory deficits are variable.
8 D’s of stroke care
Detection
Dispatch
Delivery
Door ie ED care
Data ie CT, assessment
Decision to do what
Drug or device ie thrombectomy
Disposition
Stroke care targets
Door to needle < 60 minutes in 85%
Door to device in 90 minutes for direct arrivals to ED or 60 minutes for transfer in
Stroke care targets
Door to needle < 60 minutes in 85%
Door to device in 90 minutes for direct arrivals to ED or 60 minutes for transfer in
4D’s of delay for STEMI care
Door to data (ECG)
Data to Decision
Decision to Drug or device
- Door
- Data
- Decision
- Drug
Time targets for stroke
ED assessment < 10 minutes
Neuro assessment < 20 minutes
CT/MRI done & reported < 45 minutes
Fibrinolysis < 45 min from arrival
Or
Thrombectomy < 90 minutes for direct arrivals & < 60 min for transfer in
Admission to stroke unit < 3 Hours
IV Alteplase
Plaminogen activator, human
Dose 9 mg iv bolus in 1 minute and
81 mg infusion over 1 hour
- for > 18 and also > 80 years
- for severe stroke indicated
- for mild stroke recommended
- if BP <180/110 or when lowered to this
-
Alteplase contraindications
- ICH
- Recent head injury < 3 months
- Severe ischemic large stroke with clear hypo attenuation
- ischemic stroke < 3 months
- mild disabling stroke 3-4.5 hours
- intra spinal surgery < 3 months
- structural GI malignancy
- recent bleeding event < 21 days
- coagulopathy: INR >1.7, aPTT>40, PT > 15, platelets < 100,000
- full dose LMWH taken last 24 hours
- pts on thrombin inhibitors or Factor Xa inhibitors with coagulopathy
- along with Abciximab
