Motor Neuron Disease

Question 1

what is MND

Answer 1

untreatable rapidly progressive neurodegenerative condition

Question 2

sensory symptoms are present in MND, true or false

Answer 2

False, it is MOTOR neuron disease

Question 3

what % of cases are sporadic and familial

Answer 3

90% sporadic

10% familial

Question 4

what is the peak age for MND

Answer 4

50-75 yo

Question 5

which classification of MND is most commonly used

Answer 5

UMN vs LMN

Question 6

what does PLS stand for

Answer 6

primary lateral sclerosis

Question 7

what does ALS stand for

Answer 7

amyotrophic lateral sclerosis

Question 8

what does PMA stand for

Answer 8

progressive muscular atrophy

Question 9

what does FTD stand for

Answer 9

frontotemporal dementia

Question 10

what does PBP stand for

Answer 10

progressive bulbar palsy

Question 11

PLS is UMN/LMN

Answer 11

UMN

Question 12

ALS is UMN/LMN

Answer 12

it is mixed

Question 13

PMA is UMN/LMN

Answer 13

LMN

Question 14

what is the most common type of MND

Answer 14

ALS

Question 15

UMN disease features

Answer 15

hypertonia 
hyper reflexia 
plantar reflex 
spasticity 
jaw jerk reflex

Question 16

LMN disease features

Answer 16

hypotonia 
hypo/absent reflexes
muscle wasting 
muscle weakness 
fasciculations 
floppy

Question 17

cognitive impairment is a feature of MND, true or false

Answer 17

true

Question 18

what is split hand syndrome and what is a pattern of

Answer 18

preferential wasting of thenar group muscles seen in ALS

Question 19

describe bulbar variant of MND

Answer 19

always generalises into ALS

breathing, speech, swallowing problems

Question 20

how can MND present

Answer 20

in any way - there is no classical pattern 
mixed UMN and LMN disease
dementia 
frontal 
bulbar presentation 
cachectic
tongue fasciculation 
spinal dysfunction

Question 21

MND is a diagnosis of exclusion, true or false

Answer 21

true

Question 22

what other conditions should be excluded

Answer 22

MS
Gullain Barré
multifocal motor neuropathy - MMN
Kennedy's
inclusion body myositis 
radiculopathy

Question 23

supportive management of MND

Answer 23

healthcare team
communication needs eg voice banking
nutrition - dietician, gastrostomy
ventilation

Question 24

medical management of MND

Answer 24

riluzole
many side effects
taken as a last resort

Question 25

what are respiratory red flags

Answer 25

SOB
orthopnoea 
chest infection 
disturbed sleep 
nightmares
daytime sleepiness