motor neuron disease W2

Question 1

most common form of MND?

Answer 1

amyotrophic lateral sclerosis (ALS)

Question 2

overview of MND?

Answer 2

rapidly progressive and fatal disorder
progressive neurodegeneration of upper and lower motor neurons
leads to progressive weakness, limb weakness bulbar symptoms, respiratory failure
50% have cognitive/behavioural change
15% have dementia

Question 3

time to reach diagnosis for MND?

Answer 3

1 year

Question 4

average survival of MND?

Answer 4

18 months to 3 years
30% die within 1 year
5-10% survive over a decade

Question 5

risk factors for MND?

Answer 5

male sex
increasing age
hereditary disposition (10-15% have autosomal dominant aetiology)

Question 6

pathology of classical MND?

Answer 6

gross atrophy of the primary motor cortex
thinning of atrophic anterior roots
degenerating anterior horn cells containing eosinophilic ‘bunina bodies’
accumulation of TDP-43

Question 7

what neuroanatomical pathways are affected in MND?

Answer 7

motor cortex
corticospinal tracts
anterior horn cells
motor neurons

Question 8

MND subtypes?

Answer 8

amyotrophic lateral sclerosis (60-70%)
progressive bulbar palsy (10-20%)
progressive muscular atrophy (10%)
primary lateral sclerosis (2%)
frontotemporal dementia overlap

Question 9

common symptoms of MND?

Answer 9

muscle wasting and weakness
fasciculation (twitching)
cramps
spasticity (stiffness)
breathing problems
speech and swallowing problems
problematic saliva
weight loss
fatigue
pain
cognitive and behavioural change

Question 10

UMN and LMN meaning?

Answer 10

UMN - upper motor neuron
LMN - lower motor neuron

Question 11

limb onset - UMN signs?

Answer 11

weakness
Babinskis sign
Hoffmans sign
spasticity

Question 12

limb onset - LMN signs?

Answer 12

weakness
muscle atrophy
fasciculations

Question 13

bulbar onset - UMN signs?

Answer 13

slow speech
palmomental reflex
jaw jerk reflex

Question 14

bulbar onset - LMN signs?

Answer 14

facial weakness
low palatal elevation
dysarthria
tongue fasciculations
tongue atrophy

Question 15

fasciculation meaning?

Answer 15

twitching

Question 16

spasticity meaning?

Answer 16

stiffness

Question 17

babinskis sign?

Answer 17

stimulation of lateral plantar aspect of foot leads to extension of big toe

Question 18

hoffmans sign?

Answer 18

hold out arm, palm face down, extend fingers. flick middle fingernail - if thumb or forefinger move then hoffman sign result is positive

Question 19

palmomental reflex?

Answer 19

involuntary contraction of mentalis muscle in chin caused by stimulation of the thenar eminence

Question 20

jaw jerk reflex?

Answer 20

tests patients trigeminal nerve
stretching of masseter muscle causes reflex contraction