Neurology Flashcards
Symptoms of MS
- Hemisensory loss
- Hemiparesis
- Impaired VA
- Ataxia, dysarthria
- Fatigue
- Bulbar symptoms
- Sexual dysfunction
- Vertigo
- Band sensation trunk
- Bilateral limb paraesthesia / UMN signs secondary to spinal cord involvement
- Cognitive dysfunction
No cortical signs such as aphasia and neglect
MRI MS Regions
- Juxtacortical
- Corpus callosum
- Periventricular
- Spinal cord
- Infratentorial (brainstem / cerebellar)
CSF findings
- Oligoclonal bands
- <50 weight cells
- Increased protein
Macdonald criteria
MS prognosis
50% with RRMS will progress to secondary progressive MS within 10 years
Management MS
Supportive:
- treatment of bladder dysfunction
- Botox or baclofen for spasticity
- Physiotherapy
- Occupational therapy for gait aids
- Vitamin D supplementation
- Treat sexual dysfunction
- Psychotherapy for mood
Immunosuppresive:
- IRT: Cladrabine, Alemtuzumab
- Natalizumab
- Sphingolomod
- Glataramer acetate
- Interferon
Management Acute Relapse of MS
- Prednisolone
- Methylprednisolone if severe or involving vision
- Plasmapharesis / IVIG if failing steroids
MS in Pregnancy
Glataramer acetate safe to continue
Safe to continue IRT (alemtuzumab and Natalizumab)
Most patients disease will improve during pregnancy
Myasthenia Clinical Features
- Occular symptoms (diplopia)
- Limb weakness
- Bulbar: dysarthria, dysphagia, choking
- Neck weakness
Most common acute polyneuropathy
Gullian Barre Syndrome
Gullian Barre Features
- Ascending motor sensory paralysis
- Acute onset
- Associated autonomic instability
- Preceding infective aetiology (gastro or Resp)
- Bulbar involvement
- Associated dysautonomia
*Nadir within 2-4 weeks
Investigations GBS
- Lumbar puncture raised protein no WCC
- Immune stimulus: monospot, stool MCS, HIV
- FEV1 / Vital capacity
- Nerve conduction: conduction block, increased distal latencies
- Antibodies: GQ1B in Miller fisher
Differential diagnosis for ascending paralysis
- GBS
- CIDP (Chronic mainly sensory symptoms)
- Botulism
- Diphtheria
- PAN
Seizure driving restrictions
- Commercial licence require 10 year seizure free period
- Recreational: 6-12 months seizure free period
Myasthenia Facies
Cranial Nerve Exam tips
-Diplopia : if present test whether monocular or binocular.
- Saccades: delay or slow movement, overshooting / undershooting, dysconjugate movements
- Nystagmus: fast fase is name of nystagmus, slow phase is side of pathology
- Examining 9&10: 9 is sensory 10 is motor. Test palatal movement, sensation with spatula, character voice and cough.
Cranial Nerve Exam tips
-Diplopia : if present test whether monocular or binocular.
- Saccades:
Gaze evoked nystagmus
Nystagmus present in fast fase of direction of gaze. Typically cerebellar in origin.
Monocular nystagmus
Indicated likely weakness of opposite eye ie. cranial nerve III, IV, VI palsy
Peripheral nystagmus
- Generally horizontal
- May be torsional
- Associated with corrective saccade with head impulse test
Central Nystagmus
- Vertical nystagmus
- Torsional nystagmus
- Associated with skew
Multidirectional nystagmus
- In a Gaze evoked pattern in suggestive of cerebellar disorder
- Ddx drug toxicity i.e. anticonvulsants
Upbeat Nystagmus
Central cause
- Cerebellar / medullary lesion
- Wernickes encephalopathy
- Drug intoxication
Uvula deviation
Away from lesion
Tongue deviation
Towards side of lesion
Eye Exam Proforma
Strabismus
One and half syndrome
Caused by dorsal pontine lesion.
Horizontal gaze palsy (inability to look left or right) + INO
Aetiology Horners syndrome
- Carcinoma lung apex
- Thyroid malignancy
- Neck trauma
- Lateral medullary syndrome
- Carotid artery dissection / aneurysm
- Retro-orbital lesions
Causes of anosmia
- Upper respiratory tract infection
- Meningioma olfactory groove
- Head trauma fracture cribriform plate
- Meningitis
- Kallmanns syndrome
Causes of accomodation without light reflex
- Midbrain lesion (argyll robertson pupil)
- Ciliary ganglion lesion (Adies pupil)
- Parinauds syndrome
Causes of absent accomodation but intact light reflex
Cortical lesion (cortical blindness)
Causes of pupil constriction
- Horners syndrome
- Argyll Robertson pupil
- Narcotics
- Old age
Causes of pupil dilatation
- Drugs: amphetamines, cholinergic (atropine)
- CN III palsy
- Adies pupil
- Trauma / cerebral death
- Congenital
Adies pupil
Dilated pupils that demonstrate light-near dissociation (i.e. constrict with accommodation but not light)
aetiology: damage to postganglionic parasympathetic nerve fibers secondary to viral infection
Common visual field deficits
Argyll. robertson pupil
Constricted pupils
Light-near dissociation
aetiology:
- Syphilis
- Diabetes
- alcohol
Damage to pre-tectal nuclei in midbrain
Causes of Papilloedema
- Space occupying lesion
- Meningitis
- Idiopathic intracranial hypertension (OCP, Hypertension)
- Venous sinus thrombosis
Causes of optic neuropathy
- Multiple sclerosis
- Toxic: ethambutol, alcohol, chloroquines
- Metabolic: B12 deficiency
- Ischaemia: DM, Temporal arteritis
- Familial: lebers
Causes of cataract
- Senile cataracts
- Diabetes Mellitus
- Myotonic dystrophy
- Steroids
- Trauma
Causes of ptosis
Without pupil dilatation:
- Senile ptosis
- Myasthenia gravis
- Fascioscapuhumeral dystrophy
- Myotonic dystrophy
- Thyrotoxic myopathy
Constricted pupil:
- Horners syndrome
- Tabes dorsalis
Dilated pupil:
- III nerve palsy
Occulomotor nerve palsy aetiology
Central:
- Brainstem infarction
- Tumour
- Demyelination
- Trauma
Peripheral:
- PCA aneurysm
- Raised intracranial hypertension
- Cavernous sinus lesions
VII Palsy (facial)
Uppermotor
- Vascular
- Tumour
Lower motor neurone lesion:
- Idiopathic (bells)
- Ramsay Hung (VSV)
- Parotid tumour
- CPA tumour compression
- Pontine stroke
- Multiple sclerosis
- Otitis media
- Temporal bone fracture
Bilateral lower motor neurone facial weakness
- Gullian barre syndrome
- Sarcoidosis parotid disease
- Mono-neuritis multiplex
Causes of sensoroneurial hearing loss
- Presbycusis
- SSNHL
- Trauma
- Toxic (Etoh, streptomycin)
- Infection: congenital rubella/ syphilis
- Acoustic neuroma
Causes of conductive hearing loss
- Wax
- Otitis media
- Otosclerosis
- Pagets disease
Pseudobulbar Palsy
Bilateral upper motor neurone lesions of CN 9, 10 and 12
Aetiology:
- Stroke
- MND
- Mass
- Multiple sclerosis
Bulbar vs pseudo bulbar palsy
Aetiology Bulbar palsy
- Brainstem stroke
- MS
- Myasthenia Gravis
- Syringobulbia
- Brainstem mass
- Gullian barre
Arnold Chiari Malformation
Protrusion of the cerebellar tonsils through the Forman magnum.
Features:
- Lower CN Palsies 9-12
- Cerebellar symptoms: gait ataxia
- Headache
- Myelopathy
Differential for multiple cranial nerve palsies
- Chronic meningitis
- Gullian Barre syndrome
- Brainstem lesions
- Arnold-Chiari malformations
- Trauma
- Pagets
Higher Centers Examination
Examination of dysphasia
Gertsmanns Syndrome
Damage to angular gyrus on dominant parietal lobe.
Features:
- Acalculia
- Agraphia
- Left/Right disorientation
- Finger agnosia
Primitive reflexes
-Palmomental
- Grasp
- Pout
Gait apraxia
Cortical sign / particularly frontal lobe.
Patients act as if glued to floor.
Seen in Dementia, Frontal lobe CVA
Dressing apraxia
Non-dominant parietal lobe
Types of aphasia
Dysarthria Differential
- Cerebellar dysfunction
- Pseudobulbar palsy (speech sounds hollow, and slow)
- Bulbar palsy (nasal high pitched speech, imprecise articulation)
- Myopathies
Causes of pronator drift
1) Downward drift: typically UMN weakness
2) Upward drift: hypotonia associated with cerebellar lesion
3) DCMLT loss: impaired proprioception may result in drift in either direciton
Hoffmans reflex
Sign of hypereflexia
Scrape DIP of middle finger and observe for flexion of the thumb
Causes of upper limb weakness
Upper motor neuron:
- Stroke
-MND
- Cervical myelopathy
Lower motor neurone:
- Peripheral neuropathy
- Polyneuropathy
- Radiculopathy
- Plexopathy
- MND
- NMJ disorder
Fascioscapulohumeral mucular dystrophy
- Autosomal dominant
- Onset 15-30 yoa
Features:
- Asymmetric muscle weakness of face, shoulder girl and distal leg (particularly foot drop)
- Associated hearing loss
High Steppage Gait
- Cerebellar problem
- Proprioceptive problem
Lower limb Dermatomes
Upper limb dermatomes
Upper motor neuron weakness
- Weakness predominately pyramidal pattern ie. UL extensors and LL flexors
- Hypereflexia
- Increased tone
- Clonus
- Spasticity
Peripheral neuropathy Causes
- Drugs: isoniazid, vincristine, cisplatin, amiodarone
- Toxins: Alcohol
- Amyloidosis
- Metaboli: DM, Uraemia, Hypothyroidism
- Immune: GBS / CIDP
- Vitamin B12 deficiency
- Connective tissue disease: SLE, Vasculitis, PAN
- Hereditary: CMT
Motor dominant Peripheral Neuropathy
- CMT
- Guillian Barre / CIDP
- DM
- Multifocal motor neuropathy
Sensory predominant peripheral neuropathy
- Diabetes
- Paraneoplastic asco with lung / breast Ca
- Paraproteinaemia assoc with MM
- Sjogrens
- B12 deficiency
- idiopathic
Painful peripheral neuropathy
- Diabetes
- Alcohol
- B12 deficiency
Nerve Conduction tests for peripheral neuropathy
Demyelinating:
- Aet: CIDP, MM, Diabetes
- Prolonged distal latencies, reduced conduction velocity
Axonal:
- Aet: Diabetic, ischaemic, metabolic, paraneoplastic
- Reduced velocity and amplitude
Causes of mono neuritis multiplex
- Diabetes Mellitus
- Vascilitis (PAN)
- CTD: SLE / RA
- Sarcoidosis
- Acromegaly
- HNPPP
Causes of thickened nerves
- HMSP
- Acromegaly
- CIDP
- Amyloidosis
- Sarcoidosis
- Neurofibromatosis
Causes of fasiculations
- Benign idiopathic fasiculation
- Motor neurone disease
- Spinal muscular atrophy
- Any motor neuropathy
- Motor root compression
Hereditary Sensory Motor Neuropathy
Aka Charcot Marie Tooth
Autosomal dominant
Features:
- Pes Cavus
- Hammer toes
- Motor predominant weakness affecting lower limbs and lower arms (rarely proximal to elbow or knee)
- Absent reflexes
- Slight to no sensory loss in the limbs
Brachial Plexus finding
Complete:
- LMN weakness whole arm
- Absent sensation whole arm
- May be Horners syndrome
Upper trunk (Erb) C5-6:
- Waiters tip
- unable to flex elbow, lack of shoulder movement
- Loss sensation lateral arm
Lower trunk (Klumpkes) C8-T1:
- Claw hand with paralysis of intrinsic hand muscles
- Horners syndrome
- Sensation loss medial hand and forearm
Median Nerve Palsy
Findings:
- Weakness of LOAF
- Hand of benediction
- Sensory lose lateral hand
- * Test using Pen touch or Ok sign or Oschners clasping test
Aet (Carpal Tunnel Syndrome)
- Idiopathic
- Rheumatoid arthritis
- Amyloidosis
- Hypothyroidism / Acromegaly
- Pregnancy
- Trauma
Ulnar Nerve Palsy
Findings:
- Weakness of intrinsic hand muscles (particularly finger Ab/Adductors)
- Weakness thumb adduction
- Partial Claw hand
- Sensory loss medial hand
Intrinsic Hand Muscle Wasting Differential
Nerve lesion:
- Median / ulnar neuropathy
- Brachial plexopathy
- HMSN (CMT)
Anterior horn cell:
- Motor neurone disease
- Spinomuscular atrophy
Myopathy:
- Myotonic dystrophy
Spinal cord lesions:
- Syrinogmyelia
Femoral nerve lesion
Nerve roots L2,3,4
- Weakness of knee extension
- Loss of knee jerk
- Sensory loss to inner thigh and leg
Sciatic Nerve lesion
L4-S2
- Weakness knee flexion
- Weakness to all muscle below knee -> foot drop impaired eversion/inversion
- Loss off ankle jerks
- Intact knee jerks
Common peroneal nerve palsy
- Foot drop with impaired eversion
- Sensory loss to dorsum of foot
- Intact reflexes
Causes of foot drop
- Common peroneal palsy
- L4/5 radiculopathy
- Sciatica
- HMSN
- Motor neurone disease will have hyperreflexic ankle jerk
Causes of paraplegia
- Spinal cord compression Make sure you look for spinal sensory level
- Transverse myelitis
- Anterior spinal artery occlusion (dorsal column spared)
- Multiple sclerosis
- Intrinsic cord lesion ie. syringomyelia
- Motor neuron disease
- Hereditary spastic paraplegia
Spinal Cord Lesion Signs depending on level
Above C5:
- UMN in arms and Legs
- Above C4 results in diaphragm paralysis
C5-8:
- Lower motor neurone proximal arms not hands
- UMN in lower limbs
C8-L3
- Lower motor in arms
- UMN in Limbs
- May have sensory level on trunk
Below L3:
LMN in lower limbs
Subacute combined degeneration of the cord
Features:
- Symmetrical loss of vibration and proprioception
- Sensory ataxic gait
- Upper motor neuron signs in lower limbs
- Absent ankle reflexes but extensor plantar response*** unique
- Optic atrophy
- Dementia
Brown-Sequard Syndrome
Features:
- Ipsilateral UMN weakness below level
- Ipsilateral LMN weakness at level
- Ipsilateral DCMLT loss
- Contralateral STT loss
Aetiology:
- Multiple sclerosis
- Trauma
- Glioma
Causes of spinothalamic loss only
- Central cord syndrome secondary to syringomyelia *at late stages may involve Corticospinal or DCMLT
- Anterior cord syndrome secondary to anterior spinal artery thrombosis however will also involve corticospinal tract
- Lateral medullary syndrome
Dorsal column only
- subacute combined degeneration
- MS
- Tabes dorsalis
- Peripheral neuropathy ie. diabetes
Syringomyelia features
Triad of:
1) Loss of STT in band / cape like distribution
2) LMN weakness in upper limbs
3) Upper motor neurone weakness lower limbs
Causes of proximal muscle weakness
- Myopathy
- Neuromuscular junction: MG
- Neurogenic: motor neuron disease, polyradiculopathy
Causes of Myopathy
- Muscular dystrophy
- Myotonic dystrophy
- Inflammatory myopathies
- Osteomalacia
- Alcohol
- Endocrine: Hypothyroidism, bushings, acromegaly)
- Drugs: Steroids
Muscular Dystrophies
Duchenne
- XLR
- Males only (Female with turners)
- Severe early proximal weakness rapidly progressive
- Associated with dilated CM
Beckers:
- Same as Duchenne but less rapidly progressive
Fascioscapulohumeral Dystrophy:
- Proximal shoulder girdle and muscles of fascial expression first
- Also associated foot drop
Tests for Myopathy
- CK
- EMG
- ECG -> exclude DCM
- Muscle biopsy
- Echocardiogram
Myotonic Dystrophy
Features:
- Hatchet Facies
- Wasting of muscles of facial expression, ptosis
- Fronting balding
- Weakness and myotonia (inability to relax)
- Check hand grip inability to relax
- Persussion myotonia (percuss thenar muscles watch for thumb abduction)
**
- Associated Cardiomyopathy
- Cataracts
- Diabetes mellitus
Patterns of Gait Abnormality
- Hemiparetic Gait (foot is plantar flexed and leg swung in a lateral arc)
- Paraparetic Gair (Scissor Gait)
- Parkinsonian Gait
> Shuffling
> Freezing
> Festination - Cerebella / ataxic gait: wide based staggering to affected side
- Apraxic gait: feet glued to the floor
- High Steppage gait: indicates distal weakness
- Waddling gait: indicates proximal weakness
Causes of Cerebellar disease
Unilateral:
- Stroke
- MS
- SOL: tumour / abscess
- Trauma
Bilateral:
- Drugs: phenytoin
- Alcohol
- Multiple sclerosis
- Arnold-chiari malformation
- Fredrichs ataxia
- Hypothyroidism
Cerebellar signs
- Intention tremor
- Hypotonia
- Upper limb drift
- Nystagmus
- Dysarthria
- Dysmetria
- Dysdiadochokinesis
- Ataxic gait
- Pendular knee jerks
Causes of Pes Cavus
- CMT
- Fredrichs Ataxia
Features of Freidrichs ataxia
- Bilateral cerebellar sings
- Posterior column loss in limbs
- UMN signs in the limbs
- Peripheral neuropathy
- Optic atrophy
- Pes cavus foot deformity
- Cardiomyopathy
- Diabetes Mellitus
- Autosomal Recessive*
Causes of spastic ataxia and ataxic paraparesis (upper motor and cerebellar signs combined)
Young patients:
- Spinocerebellar degeneration (Freidrichs Ataxia)
Adults:
- Multiple sclerosis
- Spinocerebellar ataxia
- Arnold chiari malformation
Differential of tremor
Resting tremor
- Parkinsons
Action tremor
- Thyrotoxicosis
- Anxiety
- Drugs
- Familial
- Idiopathic
Intention tremor Increases at end of movement
- Cerebellar disease
Causes of Parkinsonism
- Idiopathic Parkinsons
- Vascular parkinsonism
- Parkinsons plus
> MSA
> PSP
> CBD
> Lewy body - Drugs:
Antipsychotics, methyldopa
Chorea
Involuntary movements
Hemiballismus:
- Unilateral
- Lesion to subthalamic nucleus on opposite side
Athetosis:
- Slow distal writing movements.
- Lesion to putamen
Causes of Chorea
- Huntingtons disease
- Syndenhams chorea (Rheumatic Fever)
- Senility
- Wilsons
- Drugs: phenytoin, Levodopa
- SLE
Patterns of small muscle wasting of hand
Abductor polices brevis -> Median Nerve
First dorsal interossei and Abductor digiti minimi -> Ulnar nerve
Median neuropathy at carpal tunnel vs elbow.
Entrapment at wrist: mainly weakness of APB
Entrapment at elbow: also weakness of lateral two deep finger flexors (Hand of Benediction)
Investigations for Peripheral Neuropathy
- NCS / EMG to clarify axonal vs demyelinating pattern
- Nerve biopsy
- CSF to exclude CIDP
- SPEP (MM)
- ANA, ESR, CRP , ANCA
- B12 / Folate
- HbA1c
Hereditary spastic paraparesis findings
- Bilateral lower limb spasticity, clonus, hypereflexia without motor weakness or sensory loss
Types of sacaddes
Hypometric / bunny hopping → Parkinson’s disease
Corrective soccades → cerebella I vestibular disorder.
Vertical diplopia vs. Horizontal diplopia
Vertical→ cn 3 or 5
Horizontal - C N7
Describing aphasia
• Fluent vs. Non-fluent.
Repetition
Comprehension
