motor neurone Flashcards

1
Q

in MND where is there loss of motor neurones?

A

motor cortex
cranial nerve nuclei
anterior horns on spinal cord

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2
Q

what type of dementia does MND have a link too?

A

fronto-temporal

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3
Q

what is the primary symptom in MND?

A

muscle weakness

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4
Q

what is the pattern of weakness in MND?

A

usually focal and progressively spread to become generalised

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5
Q

what other symptoms are there in MND?

A

fascinations
mixed UMN and LMN symptoms
wasting of small hand muscles and tibialis anterior common

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6
Q

where does MND most commonly affect first?

A

limbs first - upper limb

can start in bulbar or thorax muscles

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7
Q

what motor neurones are most commonly degenerated first?

A

lower motor neurones then upper motor neurones (patients commonly present with a mix)

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8
Q

how can MND be distinguished from other neurological conditions?

A

doesn’t affect the eyes (myasthenia)

no sensory loss or sphincter dysfunction (MS and neuropathies)

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9
Q

what is the most common type of MND?

A

amyotrophic lateral sclerosis

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10
Q

what is the presentation of amyotrophic lateral sclerosis?

A

mixed motor neurone deficit - LMN in arms and UMN in legs

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11
Q

where does amyotrophic lateral sclerosis start?

A

starts in hands before spreading to affect all the limbs and bulbar/thorax muscles

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12
Q

what other symptoms are associated with amyotrophic lateral sclerosis?

A

weak hand grip
weak shoulder abduction
stumbling gait
foot drop

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13
Q

which type of MND has the worst prognosis?

A

progressive bulbar

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14
Q

what are the symptoms associated with progressive bulbar MND?

A

drooling
dysphagia
progressive dysarthria

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15
Q

what are the symptoms seen with progressive spinal muscular atrophy MND?

A

similar to amyotrophic lateral sclerosis but purely LMN

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16
Q

what is primary lateral sclerosis MND?

A

rare form - loss of benz cells in the motor cortex

17
Q

what symptoms are seen with primary lateral sclerosis MND?

A

purely UMN

18
Q

how is MND diagnosed?

A

clinical

nerve conduction studies to rule out other neuropathies - normal in MND

19
Q

what can be used to treat MND? which type is in mainly used in?

A

riluzole

mainly in ALS

20
Q

how long does riluzole prolong a MND patient’s life by?

A

3 months

21
Q

what other management can be used to help treat MND?

A

analgesia
anti-cholinergic for drooling
BiPAP at night for breathing
baclofen to relax muscles

22
Q

what is Kennedy’s disease?

A

may be misdiagnosed as MND

lower motor neurone limb and bulbar weakness

23
Q

what is the inheritance of Kennedy’s disease?

A

x-linked genetic disorder

24
Q

what other symptoms are associated with Kennedy’s disease?

A

erectile dysfunction
infertility
gynaecomastia
atrophic testes