motor neurone disease

Question 1

what

Answer 1

umbrella term encompassing diagnoses

progressive dysfunction of motor neurones

Question 2

common types

Answer 2

amytrophic lateral scelrosis
progressive bulbar palsy
progressive muscular atrophy
primary lateral sclerosis

Question 3

pathophysiology

Answer 3

progressive degeneration of both upper ad lower motor neurones

sensory neurones are spared

Question 4

risk factors

Answer 4

genetic component
FHx
smoking
exposure to heavy metals and certain pesticides

Question 5

typocal patient

Answer 5

late middle aged man, possibly with affected relative

insidious progressive weakness of muscles
clumsiness
slurred speach

Question 6

signs of lower motor neurone disease

Answer 6

muscle wasting
reduced tone
fasciculations
reduced reflexes

Question 7

signs of upper motor neurone disease

Answer 7

inc tone or spasticity
brisk reflexes
upgoing plantars

Question 8

Mx

Answer 8

riluzole

NIV at home
MDT
end of life care planning

Question 9

riluzole

Answer 9

can slow progression of disease

and extend survival by few months

Question 10

edavarone

Answer 10

used in US but not liscenced in UK

potential to slow disease progression