Neurology: Motor pathologies Flashcards
MND cause
Degeneration of neurones in anterior spinal cord / brain stem
MS cause
Autoimmune: chronic inflammatory CNS demyelination
MG cause
Autoimmune: chronic disorder of post-synaptic membrane at NMJ (Ab blockage of Ash Ys)
GBS cause
Autoimmune (post-infection): attack of Schwann cells on both sensory and motor nerves
Muscular dystrophy cause
Genetic (X-linked, auto dom, auto rec): progressive weakness and breakdown of muscles due to muscle protein deficiencies e.g. dystrophin
MND presentation
FADE AWAY: Fasciculations Atrophy Difficulty in speech / breathing (bulbar involvement) Early resp involvement = poor prognosis
Absence of sensory sx
Weakness
Age 50-60
Yes: rapid progression
MS presentation
Charcot’s neurologic triad: EYE= nystagmus, optic neuritis, MOUTH= dysarthria, dysphagia (Brainstem), HAND (motor tracts)= intention tremor, ataxia
Sensations: peripheral neuropathy, burning, Lhermitte’s sign
Autonomic: UI, BI, memory problems
MG presentation
Fatiguability
Muscle weakness
Diplopia
Ptosis
Dysphagia / Dysarthria
SOB (=crisis)
Facial paresis
GBS presentation
Progressive symmetrical muscle weakness
Areflexia
Resp distress
CN involvement: dysarthria, facial droop, fixed / dilated pupils
Muscular dystrophies presentation
Progressive muscle weakness
Atrophy
Scoliosis
Incoordination
Waddling gait
Resp dysfunction
Cardiomyopathy
MND investigations
Nerve conduction studies
MRI (normal)
EMG (electromyography)
MS investigations
MRI (hyper-intensities = white matter plaques)
LP (^ Abs - inflammatory)
FBC, metabolic, TFT, B12
Visual evoked potential
MG investigtaions
MuSK Abs (subtype of MG)
Serum AChR Ab analysis
Electromyography
PFT + CT thorax
Tensillon test
EMG repetitive nerve stimulation
Ice pack test
GBS investigations
LP (^ protein, normal lymph = albuminocytological dissociation)
EMG
Nerve conduction studies
Antiganglioside Ab (Miller-Fisher GBS)
Serology
Spirometry
LFT
Muscular dystrophy investigations
Genetic testing
Muscle biopsy
Blood tests (CK ^)
EMG
MND management
Supportive care (PEG feed)
BiPAP
Riluzole
MS management
Methylprednisolone
Plasma exchange
Immunomodulation
Pain relief (baclofen, gabapentin, PT)
Oxybutynin (UI)
Propanolol (tremor)
MG management
Emergency: IV Ig / plasma exchange, ventilation, DVT prophylaxis, nutrition, hydration, corticosteroids, rituximab (= anticholinesterase inhibitor)
LT: pyridostigmine, immunosuppression
GBS management
IV Ig / plasma exchange
Supportive care
= self-limiting
Muscular dystrophies management
Genetic counselling
PT / SLT / resp supporrt
Quinine (myotonia)
Orthopaedic surgery
Pacemaker (cardiomyopathy)
