Neurology Flashcards
Horners syndrome signs and associated signs
Think about the pathway of the sympathetic chain
EYES: Partial ptosis and constricted pupil (reacts normally to light)
FOREHEAD: anhidrosis
CRANIAL NERVES V/IX/X: lateral medullary syndrome
HANDS: clubbing, finger abduction (lower trunk brachial plexus C8/T1 lesion)
UPPER LIMB NEUROLOGICAL EXAM: dissociated sensory loss (syringomyelia)
RESPIRATORY EXAMINATION: apical lung tumour
VOICE: hoarseness (recurrent laryngeal nerve palsy)
NECK: cervical lymphadenopathy, thyroid carcinoma, carotid aneurysm or bruit
Causes of Horners
- Brain stem lesions – vascular disease (lateral medullary syndrome), syringobulbia, tumour
- Carcinoma of the lung apex
- Neck – thyroid malignancy, trauma
- Carotid arterial lesion – carotid aneurysm or dissection, pericarotid tumour
- Retro-orbital lesions
- Syringomyelia (rare)
Signs of Lateral Medullary syndrome
Due to PICA artery
- Horner’s syndome
- Nystagmus (to the side of the lesion) (CN 8)
- Ipsilateral cranial nerve V (pain and temperature), IX and X lesions
- Ipsilateral cerebellar signs
- Contralateral pain and temperature loss over the trunk and limbs
Signs of Medial medullary syndrome
Supplied by vertebral artery and anterior spinal artery.
Medial structures
- 12th CN
- MLF - connect 3rd and 6th CN
- Medial lemniscus - continuation of posterior column tracts.
- Pyramids - continuation of the cortico-spinal tracts
Spinal Tracts
3 sensory:
- Dorsal column / Medial lemniscus - vibration + proprioception + fine touch
- Spinothalamic - pain + temp + crude touch
- Spinocerebellar - co-ordination
2 motor:
Pyramidal tracts
- Lateral corticospinal tract
- Anterior corticospinal tract
Extrapyramidal tracts -
- Rubrospinal tract
- olivospinal tract
- vestibulospinal tract
- reticulospinal tract
Differentiating bulbar and pseudobulbar palsy.
Bulbar palsy - LMN
Gag reflex – absent
Tongue – wasted, fasciculations
“wasted, wrinkled, thrown into folds and increasingly motionless”.
Palatal movement – absent.
Jaw jerk – absent or normal
Speech – nasal
“indistinct (flaccid dysarthria), lacks modulation and has a nasal twang”
Emotions – normal
Other – signs of the underlying cause, e.g. limb fasciculations.
Pseudobulbar palsy - UMN
Gag reflex – increased or normal
Tongue – spastic
“it cannot be protruded, lies on the floor of the mouth and is small and tight”.
Palatal movement – absent.
Jaw jerk – increased
Speech – spastic: “a monotonous, slurred, high-pitched, ‘Donald Duck’ dysarthria” that “sounds as if the patient is trying to squeeze out words from tight lips”.
Emotions – labile
Other – bilateral upper motor neuron (long tract) limb signs.
Types of muscular dystrophy - Signs and symptoms of each
Duchenne- X-linked recessive, dystrophin gene, unable to walk by childhood, scoliosis and intellectual disability.
Symptoms: Proximal weakness. Tip-toe gait + tightening of achilles. Protruding abdomen due to weak abdominals. Chest forward due to weak scapula muscles. Fat infiltrated bulky calves (pseudo hypertrophy). Dilated cardiomyopathy.
Becker - Same as Duchenne but milder.
Myotonic - Autosomal Dominant, Due to trinucleotide repeats, fixed sustained contraction, difficulty releasing hand grip. also have cataracts and cardiac conduction issues.
Symptoms: Narrow face, baldness, cataracts, no chin.
Facioscapulohumeral - Autosomal dominant DUX4 gene. affects face, scapula and humeral muscles first. Commonly have winged scapula, hearing loss and foot drop. Asymmetric severity. Can cause blindness.
Signs of CN3 palsy
CLINICAL FEATURES
● Complete ptosis (partial ptosis may occur with an incomplete lesion)
● Divergent strabismus (eye ‘down and out’): limited adduction and elevation
● Dilated pupil unreactive to direct or consensual light and unreactive to accommodation
Method to test if CN4 palsy co-exists with CN3 palsy
o Tilting the patient’s head to the same side as the lesion. The affected eye will intort if the lesion is intact
o Asking the patient to look down and across to the opposite side from the lesion and look for intortion
Causes of a CN3 palsy
● Central o Vascular (e.g. brain stem infarction) o Tumour o Demyelination o Trauma ● Peripheral o Compressive lesions: ▪ Aneurysm (usually on the posterior communicating artery) ▪ Tumour causing raised intracranial pressure ▪ Nasopharyngeal carcinoma ▪ Orbital lesions ▪ Basal meningitis o Infarction: diabetes mellitus, arteritis (pupil is usually spared) o Trauma o Cavernous sinus lesions
Causes of CN6 palsy
● Bilateral o Trauma o Wernicke’s encephalopathy o Raised intracranial pressure o Mononeuritis multiplex ● Unilateral o Central: ▪ Vascular ▪ Tumour ▪ Wernicke’s encephalopathy ▪ Multiple sclerosis o Peripheral ▪ Diabetes, other vascular lesions ▪ Trauma ▪ Idiopathic ▪ Raised intracranial pressure
Causes of Nystagmus
● Horizontal:
o Vestibular lesion – chronic lesions cause nystagmus to the side of the lesion
o Cerebellar lesion – unilateral disease causes nystagmus to the side of the lesion
o Internuclear ophthalmoplegia – nystagmus is in the abducting eye, with failure of adduction on the AFFECTED SIDE. This is the result of a medial longitudinal fasciculus lesion. The most common cause in young adults with bilateral involvement is multiple sclerosis; in the elderly consider brainstem infarction
● Vertical:
o Brain stem lesion:
▪ Upbeat nystagmus suggests a lesion in the floor of the fourth ventricle
▪ Downbeat nystagmus suggests a foramen magnum lesion
o Toxic – phenytoin, alcohol (may also cause horizontal nystagmus)
Signs of Progressive supranuclear palsy
- loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.
- vertical gaze palsy with intact occulocephalic reflex.
Causes of a 5th nerve palsy
● Central (pons, medulla and upper cervical cord) o Vascular o Tumour o Syringobulbia o Multiple sclerosis ● Peripheral (posterior fossa) o Aneurysm o Tumour (skull base, e.g. acoustic neuroma) o Chronic meningitis ● Trigeminal ganglion (petrous temporal bone) o Meningioma o Fracture of the middle fossa ● Cavernous sinus (associated third, fourth and sixth nerve palsies) o Aneurysm o Thrombosis o Tumour ● Other o Sjogren’s syndrome o SLE o Toxins o Idiopathic
Causes of cavernous sinus syndrome
- Tumor- Meningioma, chordoma, neuroma, pituitary adenoma, metastases, lymphoma, nasopharyngeal carcinoma, chondrosarcoma, hemangioma, neuroblastoma
- Inflammatory Disease - Tolosa-Hunt syndrome, sarcoidosis
- Trauma- Basal skull fracture, operative trauma to cavernous sinus after skull base surgery
- Vascular - Intracavernous aneurysm, carotid-cavernous fistula, cavernous sinus thrombosis
- Infection - Mucormycosis, aspergillosis, actinomycosis, nocardiosis, mycobacterium, herpes zoster.
Causes of a 7th nerve palsy
● Upper motor neurone lesion (supranuclear) o Vascular o Tumour ● Lower motor neurone lesion o Pontine (often associated with nerves V, VI): ▪ Vascular, tumour, syringobulbia, multiple sclerosis o Posterior fossa ▪ Acoustic neuroma ▪ Meningioma o Petrous temporal bone ▪ Bell’s Palsy ▪ Ramsay Hunt Syndrome ▪ Otitis media ▪ Fracture o Parotid: ▪ Tumour ▪ Sarcoid
Causes of bilateral LMN facial weakness
● Guillain-Barre syndrome
● Bilateral parotid disease (e.g. sarcoidosis)
● Mononeuritis multiplex (rare)
Causes of cranial nerve palsies
● Central
- Vascular
- Tumour
- Syringobulbia
- Motor neurone disease
- Multiple Sclerosis
● Peripheral (posterior fossa)
- Aneurysm
- Tumour
- Infections
- polio
- Chronic meningitis
- Guillain-Barre syndrome
- Trauma/fracture of facial bones/ base of skull
- Infiltrative - sarcoid/amyloid
- Arnold-Chiari malformation
8th nerve testing interpretation
RINNE’S TEST: 256-hertz tuning fork placed on the mastoid process, behind the ear, and when the sound no longer heard, placed in line with the external meatus
● Normal – the note is audible at the external meatus
● Sensorineural deafness – the note is audible at the external meatus (air and bone conduction reduced equally)
● Conduction (middle ear) deafness – no note is audible at the external meatus – negative result
WEBER’S TEST: 256-hertz tuning fork placed on the centre of the forehead
● Normal – sound is heard in the centre of the forehead
● Sensorineural deafness – sound is transmitted to the normal ear
● Conduction deafness – sound is heard louder in the abnormal ear
- Conductive hearing loss occurs when sound conduction is impeded through the external ear, the middle ear, or both.
- Sensorineural hearing loss occurs when there is a problem within the cochlea or the neural pathway to the auditory cortex.
Multiple cranial nerve palsies
- Nasopharyngeal carcinoma
- Chronic meningitis (carcinoma, tuberculosis, sarcoidosis)
- Guillain-Barre syndrome, including Miller-Fisher variant
- Brain stem lesions
- Arnold-Chiari malformation
- Trauma
- Lesion of the base of the skull
- Mononeuritis multiplex (e.g. diabetes mellitus)
- Leptomeningeal disease
- Cavernous sinus syndrome - 3,4,V1,V2.
Types of Aphasia and sites
RECEPTIVE APHASIA
- Wernicke’s area – temporal gyrus in dominant lobe
EXPRESSIVE APHASIA
- Broca’s area – frontal gyrus
CONDUCTIVE APHASIA
- Arcuate fasciculus (temporal lobe)
NOMINAL APHASIA (difficulty naming objects)
- Angular gyrus (temporal lobe) – small localised lesion
- Encephalopathy (metabolic, toxic), pressure effects from distant space-occupying lesion
- Recovery phase from any dysphasia
Types of Dysarthria (not dysphasia)
o Cerebellar speech: slurring or “scanning” (i.e. irregular and staccato)
o Pseudobulbar palsy: slow, hesitant, hollow-sounding speech with a harsh, strained voice
o Bulbar palsy: nasal speech with imprecise articulation
Causes for drift
● Upper motor neurone weakness (usually downwards owing to muscle weakness)
● Cerebellar lesion (usually upwards owing to hypotonia)
● Posterior column loss (any direction owing to joint position sense loss)
Upper limb myotomes
● Shoulder abduction: C5,6 ● Shoulder adduction C6, C7, C8 ● Elbow flexion: C5, C6 ● Elbow extension: C7, C8 ● Wrist flexion: C6, C7 ● Wrist extension C7, C8 ● Fingers extension: C7, C8 ● Finger flexion C7, C8 ● Finger abduction: C8, T1
Upper limb reflexes
● Biceps: C5, C6
● Triceps C7, C8
● Supinator C5, C6
● Finger: C8
Causes of peripheral neuropathy
DAM IT BICH
- Drugs and toxins: isoniazid, vincristine, phenytoin, nitrofurantoin, cisplatinum, amiodarone, heavy metals
- Alcohol
- Metabolic: diabetes mellitus, uraemia, hypothyroidism
- Immune-mediated: Guillain-Barre syndrome
- Tumour – lung carcinoma, multiple myeloma
- B- Vitamin B12 or B1 deficiency
- Infiltrative: amyloid
- Connective tissues disease – SLE, polyarteritis nodosa
- Hereditary - Charcot-Marie-Tooth disease
Causes of motor neuropathy
MMILPH
- Multifocal motor neuropathy / Motor neuron disease
- Metabolic - Diabetes mellitus
- Immune - Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
- Porphyria - Acute intermittent porphyria
- Lead poisoning
- Hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease)
Causes of sensory neuropathy
BS BS PICDD
- B6 intoxication
- Sjogren’s syndrome
- B12 deficiency
- Syphilis
- Paraproteinaemia / Paraneoplastic
- Inflammatory
- CTD
- Diabetes mellitus/ Drugs
Causes of Mononeuritis Multiplex
SAC DAC
o Sarcoidosis
o Acromegaly
o Carcinoma
o Diabetes mellitus
o Arthritis - Multiple compressive neuropathies,
o CTD - Polyarteritis nodosa, SLE, rheumatoid arthritis
SAC DAC
Causes of Fasciculation
- Benign idiopathic fasciculation
- Motor neurone disease
- Motor root compression
- Malignant neuropathy
- Any motor neuropathy
Symptoms of Charcot-Marie-Tooth
ADOPTS
● Absent reflexes
● Distal muscle atrophy owing to peripheral nerve degeneration, not usually extending above the elbows or above the middle one-third of the thighs
● Optic atrophy; Argyll Robertson pupils (rare)
● Pes cavus (short, high-arched feet with hammer toes)
● Thickened nerves
● Slight to no sensory loss in the limbs
Signs of brachial plexus lesions
upper, lower and complete
COMPLETE LESION
● Lower motor neurone signs affect the whole arm
● Sensory loss (whole limb)
● Horner’s syndrome
UPPER TRUNK (C5, C6) LESION
● Loss of shoulder movement and elbow flexion – hand is held in the ‘waiter’s tip’ position
● Sensory loss is present over the lateral aspect of the arm and forearm, and over the thumb
LOWER TRUNK (C8, T1) LESION
● True claw hand with paralysis of all the intrinsic muscles
● Sensory loss along the ulnar side of the hand and forearm
● Horner’s syndrome
Signs of radial nerve lesion
● Wrist and finger drop (wrist flexion normal)
● Triceps reflex loss (elbow extension loss) if lesion is above the spiral groove. Brachioradialis reflex.
● Sensory loss over the anatomical snuff box
● Finger abduction appears to be weak because of the difficulty of spreading the fingers when they cannot be straightened
Causes and signs of median nerve lesion
● Loss of abductor pollicis brevis with a lesion at or above the wrist: ask patient to abduct thumb vertically
● Sensory loss over the thumb, index, middle and lateral half of the ring finger (palmar aspect only) \
CAUSES OF CARPAL TUNNEL SYNDROME ● Idiopathic ● Arthropathy – rheumatoid arthritis ● Endocrine disease – hypothyroidism, acromegaly ● Pregnancy ● Trauma and overuse
Signs of ulnar nerve lesion
● Wasting of the intrinsic muscles of the hand
● Weak finger abduction and adduction (loss of interosseous muscles)
● Ulnar claw-like hand (a higher lesion cause less deformity, as an above-the-elbow lesion also causes loss of flexor digitorum profundus)
● Froment’s sign: grasp a piece of paper between the thumb and lateral aspect of the forefinger – the affected thumb will flex (loss of thumb adductor)
● Sensory loss over the little and medial half of ring finger (both palmar and dorsal aspects)
Causes of wasting of the small muscles of the hand
● Nerve lesions o Median and ulnar nerve lesions o Brachial plexus lesions o Peripheral motor neuropathy (including hereditary motor and sensory neuropathy) ● Anterior horn cell disease: o Motor neurone disease o Polio o Spinal muscular atrophies ● Myopathy o Myotonic dystrophy – forearms more affected than the hands o Distal myopathy ● Spinal cord lesions o Syringomyelia o Cervical spondylosis o Tumour ● Trophic disorders o Arthropathies (disuse) o Ischaemia including vasculitis
Femoral nerve lesion
● Weakness of knee extension (quadriceps paralysis)
● Slight hip flexion weakness
● Preserved adductor strength
● Loss of knee jerk
● Sensory loss involving the inner aspect of the thigh and leg
Sciatic nerve lesion
● Weakness of knee flexion (hamstrings involved)
● Loss of power of all muscles below the knee causing a foot drop, so the patient may be able to walk, but cannot stand on the toes or heels
● Knee jerk intact
● Loss of ankle jerk and plantar response
● Sensory loss along the posterior thigh and total loss below the knee
Common peroneal nerve lesion
● Foot drop and loss of foot eversion only
● Sensory loss (minimal) over the dorsum of the foot
● Normal reflexes - intact ankle jerk
Causes of foot drop
● Common peroneal nerve palsy ● Sciatic nerve palsy ● Lumbosacral plexus lesion ● L4, L5 root lesion ● Peripheral motor neuropathy ● Distal myopathy ● Motor neurone disease
What is Subacute combined degeneration of spinal cord.
Subacute combined degeneration of spinal cord, refers to degeneration of the dorsal column and lateral corticospinal tract of the spinal cord as a result of vitamin B12 deficiency (most common), vitamin E deficiency, and copper deficiency. It is usually associated with pernicious anemia.
Symptoms
- Dorsal column - loss of fine touch, proprioception, vibration. Symmetrical posterior column loss (vibration and position sense) causing an ataxic gait.
- Corticospinal tract - weakness
- Symmetrical upper motor neurone signs in the lower limbs with absent ankle reflexes
- Optic atrophy (occasionally)
- Dementia (occasionally)
Causes of positive babinski but loss of ankle jerk
Some cord syndromes confuse floundering doctors
● Subacute combined degeneration of the cord (Vitamin B12 deficiency)
● Conus medullaris lesion
● Combination of an UMN lesion with cauda equina compression or peripheral neuropathy
● Syphilis
● Friedreich’s ataxia
● Diabetes mellitus
Brown Sequard Syndrome - Causes and clinical signs
Causes: ● Multiple sclerosis ● Angioma ● Glioma ● Trauma ● Myelitis ● Postradiation myelopathy
Signs
● Motor changes
o Upper motor neurone signs below the hemisection on the same side as the lesion
o Lower motor neurone signs at the level of the hemisection on the same side
● Sensory changes
o Pain and temperature loss on the opposite side of the lesion (the upper level of the sensory loss is usually a few segments below the level of the lesion)
o Vibration and proprioception loss on the same side
o Light touch is often normal
o There may be band of sensory loss on the same side at the level of the lesion (afferent nerve fibres)
Causes of SPINOTHALAMIC (PAIN AND TEMPERATURE) LOSS ONLY
SPINOTHALAMIC (PAIN AND TEMPERATURE) LOSS ONLY
● Syringomyelia (‘cape’ distribution)
● Brown-Sequard syndrome (contralateral leg)
● Anterior spinal artery thrombosis
● Lateral medullary syndrome (contralateral to the other signs)
● Peripheral neuropathy (diabetes mellitus, amyloid, Fabry’s disease)
Causes of DORSAL COLUMN (VIBRATION AND PROPRIOCEPTION) LOSS ONLY
DORSAL COLUMN (VIBRATION AND PROPRIOCEPTION) LOSS ONLY
● Subacute combined degeneration
● Brown-Sequard syndrome (ipsilateral leg)
● Spinocerebellar degeneration (e.g. Friedreich’s ataxia)
● Multiple sclerosis
● Tabes dorsalis (neurosyphilis)
● Sensory neuropathy or gangliopathy (e.g. carcinoma)
● Peripheral neuropathy from diabetes mellitus or hypothyroidism
Syringomyelia
● Loss of pain and temperature over the neck (spinothalamic - due to decussation), shoulders and arms (‘cape’ distribution)
● Amyotrophy (weakness, atrophy and areflexia) of the arms
● Upper motor neurone signs in the lower limbs
Cyst elongates over time - more nerves effected over time.
Linked to Arnold Chiari malformation
Syringobulbia
Same as Syringomyelia but occurs in the lower brainstem.
Compress the lower cranial nerve nuclei or ascending sensory or descending motor pathways.
Causes of proximal myopathy
- Myopathic
- Neuromuscular junction disorder – myasthenia gravis
- Neurogenic – motor neurone disease, polyradiculopathy
Causes of myopathy
● Hereditary muscular dystrophy ● Congenital myopathies ● Acquired (PACE PODS) o Polymyositis or dermatomyositis o Alcohol o Carcinoma o Endocrine (hypothyroidism, hyperthyroidism, Cushing’s syndrome, acromegaly, hypopituitarism) o Periodic paralysis o Osteomalacia o Drugs (steroids, statins) o Sarcoidosis
Types of gaits
● Hemiplegic/ Circumducting - the foot is plantarflexed and the leg swung in a lateral arc - due to pyramidal weakness. Can have associated flexion in arm.
● Diplegic (scissor gait) - associated with cerebral palsy - tiptoe gait with circumduction and excessive adduction.
● Extrapyramidal (e.g. Parkinson’s Disease)
o Hesitation in starting, shuffling, freezing, propulsion/retropulsion. All joints flexed.
● Cerebellar - wide based and clumsy/staggering. Falls towards side of lesion. Can have truncal ataxia.
● Posterior column lesion (clumsy slapping down of the feet on a broad base) - to increase vibration into the truck to help proprioception.
● Distal weakness (high-stepping gait) - from foot drop
● Proximal weakness (waddling gait) - due to weakness of the pelvic girdle - leans trunk to the side to compensate. Will have positive Trendelenburg’s sign.
● Apraxic (prefrontal lobe): feet appear glued to the floor when erect, but move more easily when supine
● Antalgic gait -faster gait cycle on unaffected leg to reduce weight bearing. May tip toe on affected leg.
Causes of cerebellar disease
UNILATERAL
- Space-occupying lesion (tumour, abscess, granuloma)
- Ischaemia (vertebrobasilar disease)
- Paraneoplastic syndrome
- Multiple sclerosis
- Trauma
BILATERAL
- Drugs (e.g. phenytoin)
- Friedreich’s ataxia
- Hypothyroidism
- Multiple sclerosis
- Trauma
- Arnold-Chiari malformation
- Alcohol
- Large space-occupying lesion, cerebrovascular disease
Signs of Friedreichs ataxia
Mitochondrial condition affecting cerebellum, cardiac fn and pancreas. –> frataxin protein for mitochondrial proteins –> trinucleotide repeat.
● Cerebellar signs (bilateral) including nystagmus
● Posterior column loss in the limbs
● Upper motor neurone signs in the limbs (although ankle reflexes are absent)
● Peripheral neuropathy
● Optic atrophy
● Pes cavus, cocking of the toes and kyphoscoliosis
● Cardiomyopathy
● Diabetes mellitus from pancreas
Causes of Pes Cavus
A high arched foot with fixed plantar flexion associated with degeneration of the spinocerebellar tract.
● Friedreich’s ataxia or other spinocerebellar degenerations
● Hereditary motor and sensory neuropathy (Charcot-Marie-Tooth)
● Neuropathies in childhood
Charcot-Marie- Tooth (Hereditary motor and sensory neuropathy)
Autosomal dominant spectrum of diseases affecting the MYELIN SHEATH - therefore affects mainly peripheral nerves.
Mainly affects lower limbs below the knees.
- stork legs
- pes cavus
- hammer toes
- claw hands
- parasthesias
- loss of pain + temp
- loss of dorsal column
- onion bulb effect on nerve biopsy
Causes of spastic and ataxic paraparesis
● Multiple sclerosis ● Spinocerebellar degeneration ● Syringomyelia ● Bilateral upper pons or internal capsule infarction ● Lesion at the craniospinal junction
Causes of Parkinsonism
● Idiopathic (Parkinson’s Disease)
● Drugs (phenothiazines, methyldopa)
● Post-encephalitis
● Other: toxins (carbon monoxide), Wilson’s disease, Progressive Supranuclear Palsy, Multiple-System Atrophy, syphilis, tumour
Types of tremor
● Parkinsonism – resting tremor
● Action tremor – present throughout movement but resolves at rest
o Thyrotoxicosis
o Anxiety
o Drugs
o Familial
o Idiopathic (most common)
● Intention tremor (cerebellar disease) – increases towards the target
● Cerebellar outflow tract tremor (‘red nucleus’) – abduction-adduction movements of upper limbs with flexion-extension of the wrists
Causes of chorea
● Huntington’s disease
● Sydenham’s chorea (rheumatic fever)
● Wilson’s disease
● Drugs (OCP, phenytoin, L-Dopa)
● Vasculitis or connective tissue disease (e.g. SLE)
● Thyrotoxicosis
● Polycythaemia or other causes of hyperviscosity
One and a half syndrome
“a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other”
● Paramedian pontine lesion of paramedian reticular formation (PPRF) and/or the 6th nerve nucleus and the medial longitudinal fasciculus on the same side
Causes - Multiple sclerosis, infarction, hemorrhage, and tumour
● Turning out (extropia) of the eye opposite the side of the lesion
Miller Fischer triad
Ataxia, Areflexia, Ophthalmoplegia
Causes of conductive deafness
Wax
Otitis media
Otosclerosis
Paget’s disease of bone
Causes of vestibular symptoms
Labyrinth - acute labrinthitis - motion sickness - aminoglycaside toxicity - meniere’s
Vestibular - vestibular neuronitis
Brainstem (central connections of the vestibular system) - vascular lesions - tumors of the cerebellum or 4th ventricle - demyelination - vasospastic conditions (i.e. migraine)
Temporal lobe - ischaemia - seizures
What is Hereditary spastic paraparesis
A group of inherited diseases whose main feature is a progressive gait disorder, presents with progressive spasticity and contraction in the lower limbs.
UMN weakness/signs.
Length-dependent axonal degeneration (no demyelination) - mainly cortico-spinal tract and spinocerebellar.
dorsal column and spinothalamic also involved to a lesser extent.
Upper limb sparing/ less affected.
Signs of Huntington’s Disease
A subcortical disease - affects caudate and putamen.
Autosomal dominant due to a trinucleotide repeat.
Enlarged ventricles on MRI due to subcortical shrinking.
Is a movement disorder with dementia.
Chorea/balism + Extrapyramidal features + UMN signs + cerebellar features + psych changes.
General inspection - chorea/balism - dystonic movements - bradykinesia - dysarthria - dysphagia - involuntary vocalisations - dementia/depression/psych Tone - hypotonic early with hyperreflexia - akinetic/rigid when advanced - postural instability Power - motor impersistence (can't keep tongue protruded) Other - slowed visual saccades - may have cerebellar features in the limbs
Upper and Lower limb reflexes - nerve roots and nerve
Knee jerk - L3-4 Ankle - L5-S1 (mainly S1) Biceps - C5 Musculocutaneous Brachioradialis - C6 Radial Triceps - C7 Radial Finger flexion (index, middle) - C8 Median
Signs of Tabes dorsalis (neurosyphilis)
Features
- argyll-robertson pupil and visual impairment
- dorsolateral column degeneration (loss of vibration/proprioception, sensory ataxic gait) - with positive Rhomberg’s
- spinothalamic degeneration - parasthesia, hyperaesthesia, burning/shooting pains - back pain - hypotonia - hyporeflexia - weakness.
Features of Gertsmans syndrome (Dominant parietal lobe stroke)
Acalculia, Finger agnosia, Agraphia, L-R disorientation
Stroke causing loss of Ant 2/3 tongue taste
Marie Foix - AICA stroke - Facial nuclei involved rather than just facial nerve
Stroke syndromes:
- Contralateral weakness and sensory loss more marked in the upper limbs and lower half of the face than in lower limbs
- Gaze deviates toward the side of infarction
- Contralateral homonymous hemianopia without macular sparing
- Aphasia if in dominant hemisphere
- Broca aphasia (lesion to inferior frontal gyrus)
- Wernicke aphasia -Occurs in lesion to superior temporal gyrus
- Conduction aphasia
- Hemineglect if in nondominant hemisphere
- Sensory neglect
MCA
Stroke syndromes:
- Contralateral weakness and sensory loss in the lower limbs more marked than in upper limbs
- Abulia (lack of will)
- Urinary incontinence
- Dysarthria/ Transcortical motor aphasia
- Frontal release signs
- Limb apraxia
ACA
Stroke syndromes:
- Contralateral homonymous hemianopia with macular sparing due to occipital lobe involvement
- Contralateral sensory loss due to lateral thalamic involvement: light touch, pinprick, and positional sense may be reduced.
- Memory deficits
- Vertigo, nausea
PCA
Stroke syndromes:
- Ipsilateral bulbar palsy (dysphagia, dysphonia, hiccups, decreased gag reflex)
- Ipsilateral nystagmus and vertigo (vestibular nuclei)
- Pain and temperature - contralateral to body and ipsilateral to face.
- limb ataxia
- Horners syndrome
PICA
Stroke syndromes:
- Contralateral body pain and temp loss
- ipsilateral ataxia
- ipsilateral face pain + temp loss - 5thCN
- ipsilateral weakness - 7th CN
- Loss of anterior 2/3 tongue sensation
- Ipsilateral horners and vestibular nuclei
AICA
Stroke syndromes:
- Locked in syndrome
Basilar
Stroke syndromes:
Amaurosis Fugax
Internal carotid
Stroke syndromes:
Horner’s syndrome alone
Common carotid
Area of brain causing hemiballismus
Subthalamic nucleus
Stroke syndromes:
Contralateral hemiplegia and paresthesia
Gaze palsy and ipsilateral deviation of the eyes
Stupor and coma
Putamen
Stroke syndromes:
Contralateral hemiparesis and paresthesia
Miotic and unreactive pupils, upgaze palsy with gaze deviation away from the side of the lesion (wrong way eyes)
Thalamus
Signs of cervical rib syndrome
Lower trunk brachial plexus lesion (C8/T1) - true claw hand with sensory loss on ulnar side of hand and forearm
Unequal radial pulses and blood pressures
Subclavian bruit; loss of pulse on arm manoeuvring
Palpable cervical rib in the neck (uncommon)
Interpretation of Rhomberg’s test ?
Feet together standing upright
Cerebellar issue - eyes open lose balance, no difference with eyes closed
Dorsal column issue - eyes open ok, eyes closed lose balance
Pattern of pyramidal weakness
Upper limb - weak extensors
Lower limb - weak flexors
Cortical diseases
Stroke Tumor Demyelinating – MS Motor cortex (pre-frontal) Posterior cortical atrophy Corticobasal degeneration Alzheimers Demetia with lewy bodies Fronto-temporal dementia Neurosyphillus Cerebral palsy
Sub-cortical diseases
Parkinsons + Parkinsonian disorders. Thalamic strokes - Pure motor - Pure sensory Huntingtons Wilsons disease Progressive supra-nuclear palsy
Brainstem diseases
Midbrain syndrome – PCA Medial pontine syndrome – Basilar Lateral Pontine syndrome – AICA Medial medullary syndrome – ASA Lateral medullary syndrome – PICA
Spinal cord diseases
Syringomyelia Central cord syndrome Anterior cord syndrome Posterior cord syndrome MND (mixed UMN and LMN) Spinocerebellar ataxia Friedrichs ataxia Brown Sequard
Anterior horn cell diseases
Spinal muscular atrophy
Poliomyelitis - polio virus induced flaccid paralysis.
Amyotrophic lateral sclerosis
Nerve root diseases
Arthritis Spinal canal stenosis Spondylosis Disc herniation Spinal meningioma Neurofibroma Herpes zoster
Plexopathies
Trauma
Malignancy
Diabetes
Radiation
Peripheral nerve diseases
Diabetes B12/B6 Syphilis Sjogrens Paraprotein Charcot marie tooth (HSMN) Guillian Barre MND CTD Sarcoid
NMJ diseases
Myasthenia Gravis
- AcH antibody
- associated with thymic tumor
- Weakness worse with exercise
- Decrementation on repeated nerve stimulation
- normal reflexes and autonomic function
Lambert Eaton
- Voltage gated calcium channel antibody
- associated with SCLC
- weakness improves with exercise
- incrementation on nerve stimulation
- reduced reflexes and autonomic dysfunction
Botulism
- neuromuscular blockage causing flaccid paralysis.
Myositis types + antibodies + signs
- Inclusion body myositis
- distal upper limb weakness and proximal lower limb weakness.
-anti-CN1A
-Histo - CD8 infiltration and increase MHC1 expression.
Vacuoles - Polymyositis
- Proximal weakness
- Anti Jo - associated with antisynthetase syndrome and ILD.
- Histo - CD8 infiltration and increase MHC1 expression.
- Dermatomyositis
- Proximal weakness
- MDA5 - Rapidly progressive DM. ILD, less muscle involvement. Mi2 TIF-1 –> highest risk for malignancy.
- Necrotizing myositis
- Proximal weakness
- Anti SRP/ Anti HMGCoR
- Histo - necrotic fibres with MACROPHAGES and COMPLEMENT.
Signs:
Gottron’s papules
Poikiloderma (shawl sign)
Heliotrope rash
Features of Motor Neuron Disease
- Most common is Amyotrophic Lateral sclerosis
- Affects BOTH UMN and LMN
- pure motor disease
- usually have cranial nerve involvement - dysphagia/dysarthria.
- reduced muscle bulk.
- nasal speech
- have primitive reflexes
- bowel/bladder/occular muscles conserved.
- involuntary laughing/ crying.
- investigate with EMG
Niemann Pick disease - Pathophys and signs
Its is a lysosomal storage disease causing neurological symptoms, hepatosplenomegaly and bone marrow infiltration.
Accumulation of sphingomyelin in the central nervous system including the cerebellum.
- ataxia, dysarthria, dysphagia.
- Basal ganglia dysfunction
- Upper brainstem disease results in nystagmus and supranuclear gaze palsy.
- dementia and seizures.
Spinocerebellar ataxia
Due to a trinucleotide repeat.
Autosomal dominant
Cerebellar and spinal cord degeneration.
Central cord syndrome
- Bilateral crossing spinothalamic tracts affected = suspended area of bilateral sensory loss.
- Large lesion causes anterior horn cell compression = LMN weakness at the level of the lesion.
- Very large lesion affects corticospinal tract = UMN weakness below the level of the lesion.
Spinal Muscular atrophy - cause and signs
Autosomal recessive mutation in SMN1 gene.
Causes death of peripheral motor nerves - death at cell body which lies in the anterior horn.
Lower motor neuron signs
Scoliosis due to wasting of paraspinal muscles
Can have respiratory involvement.
