MND

Question 1

which part of the NS does motor neurone disease affect?

Answer 1

neurodegenerative condition affecting motor neurones in both the CNS and PNS, therefore causes both UMN and LMN signs on examination.

Question 2

types of MND?

Answer 2

amyotrophic lateral sclerosis-most common
progressive muscular atrophy-pure LMN features with small muscles of hands and feet generally affected 1st, but lack spasticity
Affects anterior horn cells only
progressive bulbar palsy-problems with speech and swallowing
primary lateral sclerosis-leg muscle weakness mainly, only UMN signs

Question 3

cause of MND?

Answer 3

unknown
5% of those affected have a familial form of the disease due to a mutation in superoxide dismutase-1 gene
?mitochondrial dysfunction causing oxidative stress in motor neurones

Question 4

who does MND affect?

Answer 4

more common in adults over 50 years
male to female ratio 2:1
FH

Question 5

what type of dementia do MND patients tend to develop?

Answer 5

frontotemporal

Question 6

examples of symptoms noted by the pt with limb onset of MND?

Answer 6

weakness, usually affecting upper limbs: dropping things, difficulty manipulating objects e.g. turning keys, unscrewing bottles
wrist drop, stiffness, weakness or cramping of hands
wasting of intrinsic hand muscles
fasciculations
leg problems:
foot drop
tripping
heaviness in 1 or both legs
difficulty in rising from low chairs and climbing stairs
excessive fatigue when walking

Question 7

features of bulbar onset amyotrophic lateral sclerosis?

Answer 7

dysarthria
dysphagia
wasting and fasciculation of tongue
accompanying emotional lability-inappropriate laughing or crying
drooling
choking
pulmonary aspiration

Question 8

What drug can be used to slow the progression of MND?

Answer 8

Riluzole-antiglutamate treatment