Motor Neurone Disease

Question 1

Is MND usually familial or sporadic?

Answer 1

90% sporadic

Question 2

MND usually presents in what age group?

Answer 2

> 40

Question 3

signs of MND

Answer 3

muscle weakness, stumbling spastic gait, foot drop, may be problems with swallowing and breathing

Question 4

where is the usual site of onset in MDN - bulbar, thoracic or extremities?

Answer 4

extremities - more often upper

Question 5

what is the most common form

Answer 5

ALS (amyotrophic lateral sclerosis)

Question 6

what type of dementia does ALS have a link with?

Answer 6

frontotemporal dementia

Question 7

features of ALS

Answer 7

tipping when walking, dropping things

Question 8

bulbar variant, always generalisation to ?

Answer 8

ALS

Question 9

features of bulbar variant

Answer 9

slurring of speech, difficulty swallowing

Question 10

complication of bulbar variant

Answer 10

aspiration pneumonia

Question 11

treatment of MND

Answer 11

antiglutamergic drugs - RILUZOLE

Question 12

side effects of riluzole

Answer 12

^LFT, vomiting, ^pulse, somnolence, headache

Question 13

tx for drooling

Answer 13

propantheline, amitriptyline

Question 14

tx for resp failure

Answer 14

non invasive ventilation at home

Question 15

what are the less common variants

Answer 15

progressive muscular atrophy,
progressive lateral sclerosis
progressive bulbar palsy