Motor Neurone Disease

Question 1

what is motor neurone disease?

Answer 1

untreatable and rapidly progressive neurodegenerative condition

Question 2

classification of MND

Answer 2

1. amyotrophic lateral sclerosis (ALS)
2. progressive bulbar palsy (PBP)
3. progressive muscular atrophy (PMA)
4. primary lateral sclerosis (PLS)

Question 3

what is affected in ALS?

Answer 3

UMN and LMN

poor survival

Question 4

associations with PBP

Answer 4

women 60-80

generalises to ALS

Question 5

presentation of PBP

Answer 5

bulbar dysfunction affecting tongue, facial and pharyngeal muscles

Question 6

signs in PMA

Answer 6

LMN

Question 7

signs in PLS

Answer 7

UMN

good survival

Question 8

presentation

Answer 8

insidious and progressive muscle weakness/ clumsiness
problems with speech, swallow and breathing
UMN signs (increased tone)
LMN signs (wasting and weakness)
without sensory signs
cognitive impairment
split hand syndrome in ALS

Question 9

diagnosis

Answer 9

raised CK
clinical
electrophysiology

Question 10

management

Answer 10

ongoing= key worker, communication needs, nutrition, respiratory
gastrotomy early as metabolic rate doubled
baclofen for muscle spasm
BiPAP mask if type 2 respiratory failure due to weakness of respiratory muscles