Motor neurone disease Flashcards
1
Q
what are the 4 types of MND and their main differences ?
A
amyotrophic lateral sclerosis / spinal
- UMN and LMN
- UMN upper limb & LMN in lower limb
progressive muscle atrophy
- LMN
- tends to affect distal limbs first
primary lateral sclerosis
- UMN
progressive bulbar palsy
- palsy of CN IX, X and XI
- affects tongue, muscles of chewing, swallowing, cough
2
Q
what is the genetic association with familial MND ?
A
gene responsible lies on chromosome 21 which codes for superoxide dismutase
3
Q
what type of MND does familial MND present with ?
A
ALS
4
Q
how would you diagnose MND ?
A
clinical diagnosis
may do;
- nerve conduction studies which will be normal
- EMG = reduced number of action potentials with increased amplitude
5
Q
describe the management of MND.
A
mainly supportive
- NIV, usually BIPAP, at night
- NG / PEG feeding
- pain relief
- spasticity = baclofen
Riluzole (mainly used in ALS)
6
Q
action of riluzole ?
A
prevents stimulation of glutamate receptors
