Motor Neurone Disease Flashcards

1
Q

how is MND diagnosed?

A

clinical diagnosis

can do neurophysiology to support this

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2
Q

how does MND often present?

A

muscle weakness

problems with speech, swallow and breathing

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3
Q

describe the course of disease in MND

A

focal onset

spreads until finally causing a generalised paresis if left untreated

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4
Q

what is the most common subtype of MND?

A

amyotrophic lateral sclerosis (ALS)

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5
Q

what is the average survival in MND?

A

three years

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6
Q

what four conditions are considered to be MND?

A

amyotrophic lateral sclerosis
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy

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7
Q

how does ALS present?

A

UMN and LMN features

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8
Q

how does primary lateral sclerosis present?

A

UMN features

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9
Q

how does progressive muscular atrophy present?

A

subclinical UMN features

LMN features

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10
Q

name some upper motor neurone signs that may be seen in MND

A
increased tone 
hyper-reflexia 
extensor plantar responses 
spastic gait 
exaggerated jaw jerks 
slowed movements
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11
Q

name some lower motor neurone signs that may be seen in MND

A

muscle wasting
weakness
fasciculations
absent/reduced deep tendon reflexes

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12
Q

what is one of the main causes of death in MND

A

weakness of respiratory muscles

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13
Q

what are the red flag respiratory signs in MND?

A
breathlessness 
orthopnoea 
recurrent infection 
disturbed/non refreshing sleep
daytime sleepiness 
poor concentration
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14
Q

what defines progressive bulbar palsy?

A

primary bulbar onset of symptoms

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15
Q

who is most commonly affected by progressive bulbar palsy?

A

women more commonly than men

60-80 years of age

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16
Q

what is the outcome of progressive bulbar palsy?

A

always undergoes generalisation into ALS

17
Q

what are possible therapeutic interventions for progressive bulbar palsy?

A

early communicator
nutritional support
care for the URT

18
Q

what muscles are affected by progressive bulbar palsy?

A

tongue muscles
facial muscles
pharyngeal muscles

19
Q

what is split hand syndrome and what disease is this seen in?

A

preferential wasting of the thenar muscle group

ALS

20
Q

what is the pathophysiology behind ALS?

A

motor neurone degeneration and death

21
Q

what investigation is done for ALS?

A

electrophysiology

largely a diagnosis of exclusion - neuroimaging and lab studies to exclude other diseases

22
Q

what is the diagnostic criteria for ALS?

A

the el escorial criteria

classifies into clinically definite, clinically probable, probably (lab supported) and clinically possible ALS

23
Q

what is the only drug available for MND and how beneficial is this?

A

riluzole

only adds on three months to a patients life

24
Q

what are the options for managing sialorrhoea in MND?

A

hyoscine/buscopan
glycopyronium
botox
suction

25
Q

what are the options for managing muscle cramps in MND?

A

quinine

baclofen

26
Q

what are the options for managing muscle spasms in MND?

A

baclofen
tizanidine
dantrolene
gabapentin

27
Q

what is the option for managing SOB and anxiety in MND?

A

lorazepam