Neurodegenerative Disorders Flashcards
What are the 5 areas mostly affected in motor neurone disease?
Motor cortex - precentral gyrus Corticobulbar pathway CN nuclei, particularly 7 and 9 Corticospinal tract Anterior horn cells
What is found in the anterior grey matter horn?
Motor neurons - mostly cell bodies of alpha motor neurons
Give 5 diseases that preferentially effect the anterior horn cells?
MND CMT disease Spinal muscular atrophy Poliomyelitis West Nile disease
What is the type of MND characterised by early corticospinal tract damage and thus UMNL sign predilection?
Primary lateral sclerosis
What is primary lateral sclerosis?
Early corticospinal tract damage in MND so prominent UMNL signs and pyramidal weakness
What is pyramidal weakness and what does it suggest?
Extensor weakness in ULs, flexor weakness in LLs
Indicates corticospinal or motor cortex damage
What is the ‘unit’ characteristically most affected in motor neuron disease?
Anterior horn
What type of MND is characterised by early anterior horn damage and this LMNL-sign prominence?
Progressive muscular atrophy
What characterises progressive muscular atrophy?
Anterior horn cell damage early and LMNL prominence
What type of MND has the best prognosis?
Progressive muscular atrophy
What is bulbar-onset motor neuron disease otherwise known as?
Progressive bulbar palsy
Where is the pathology in progressive bulbar palsy?
Corticobulbar pathway and lower CN motor nuclei involvement
What are the cranial nerves mostly implicated in progressive bulbar palsy?
CN 5, 7, 10, 11 and 12
What is a metabolic differential for progressive muscular atrophy?
Diabetic amyotrophy
Major common differentials for motor neuron disease that warrants spinal MRI?
Cervical spondylosis
Spinal rumours
Poisoning with what can yield an ALS-like picture?
Lead
Lysosomal storage disorder that can present like MND?
Tay-Sachs disease
3 neurological signs that do not occur in MND?
Sensory signs
Bladder disturbance
Ocular muscle involvement
What happens to conduction velocity in MND?
Normal velocities
What 2 signs does EMG show in MND?
Denervation
Fibrillation
What metabolic parameters should be checked before diagnosing MND and why?
TFTs and PTH/Ca
Because hyperthyroidism and hyperPTH can mimic with UMNL and LMNL signs
What is the only current disease modifying drug for MND and what properties does it have? How long does it add on to life expectancy?
Riluzole - anti-glutamate
Adds on maybe 2m
