Muscular Disease

Question 1

define motor neurone disease (MND)

Answer 1

a spectrum of heredeodegenerative diseases of the peripheral and central motor nerves - causing upper and lower motor neuron degeneration

Question 2

clinical features of MND

Answer 2

upper motor neuron and lower motor neuron signs

Question 3

describe upper motor neuron signs

Answer 3

spasticity
hyperreflexia
upgoing plantars

Question 4

describe lower motor neuron signs

Answer 4

fasciculations
atrophy
hypotonia

Question 5

characteristic presentation of spinal muscular atrophy

Answer 5

limb weakness due to involvement of spinal cord anterior horn cells

Question 6

characteristic presentation of primary lateral sclerosis

Answer 6

spastic limb weakness due to UMN involvement of the spinal cord

Question 7

characteristic presentation of bulbar palsy

Answer 7

early tongue and bulbar involvement - often a progressive disease

Question 8

characteristic presentation of primary lateral sclerosis

Answer 8

UMN features ONLY

Question 9

management of MND

Answer 9

antispasmodics (e.g. baclofen) 
antiglutamenergics (e.g. riluzole) 
anticholinergics (for drooling)
analgesia 
emotional and symptomatic support