Motor Neuron Disease Flashcards

1
Q

what is MND

A

untreatable rapidly progressive neurodegenerative condition

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2
Q

sensory symptoms are present in MND, true or false

A

False, it is MOTOR neuron disease

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3
Q

what % of cases are sporadic and familial

A

90% sporadic

10% familial

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4
Q

what is the peak age for MND

A

50-75 yo

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5
Q

which classification of MND is most commonly used

A

UMN vs LMN

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6
Q

what does PLS stand for

A

primary lateral sclerosis

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7
Q

what does ALS stand for

A

amyotrophic lateral sclerosis

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8
Q

what does PMA stand for

A

progressive muscular atrophy

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9
Q

what does FTD stand for

A

frontotemporal dementia

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10
Q

what does PBP stand for

A

progressive bulbar palsy

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11
Q

PLS is UMN/LMN

A

UMN

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12
Q

ALS is UMN/LMN

A

it is mixed

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13
Q

PMA is UMN/LMN

A

LMN

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14
Q

what is the most common type of MND

A

ALS

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15
Q

UMN disease features

A
hypertonia 
hyper reflexia 
plantar reflex 
spasticity 
jaw jerk reflex
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16
Q

LMN disease features

A
hypotonia 
hypo/absent reflexes
muscle wasting 
muscle weakness 
fasciculations 
floppy
17
Q

cognitive impairment is a feature of MND, true or false

A

true

18
Q

what is split hand syndrome and what is a pattern of

A

preferential wasting of thenar group muscles seen in ALS

19
Q

describe bulbar variant of MND

A

always generalises into ALS

breathing, speech, swallowing problems

20
Q

how can MND present

A
in any way - there is no classical pattern 
mixed UMN and LMN disease
dementia 
frontal 
bulbar presentation 
cachectic
tongue fasciculation 
spinal dysfunction
21
Q

MND is a diagnosis of exclusion, true or false

A

true

22
Q

what other conditions should be excluded

A
MS
Gullain Barré
multifocal motor neuropathy - MMN
Kennedy's
inclusion body myositis 
radiculopathy
23
Q

supportive management of MND

A

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

24
Q

medical management of MND

A

riluzole
many side effects
taken as a last resort

25
Q

what are respiratory red flags

A
SOB
orthopnoea 
chest infection 
disturbed sleep 
nightmares
daytime sleepiness