Motor Neuron Disease Flashcards

1
Q

what is MND

A

untreatable and rapidly processing neurodegenerative condition
- average survival = 3 years

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2
Q

symptoms of MND

A

muscle weakness + wasting
speech difficulty
swallowing / breathing difficulty
UMN + LMN signs

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3
Q

what is not affected in MND

A

no sensory or sphincter disturbance

- helps distinguish from MS/polyneuropathies

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4
Q

4 types of MND

A
  1. amyotrophic lateral sclerosis (ALS)
  2. Progressive muscular atrophy
  3. Progressive bulbar palsy + Pseudobulbar palsy
  4. Primary lateral sclerosis
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5
Q

what type of MND is most common

A

ALS

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6
Q

what type of MND only affects lower Motor neurones

A

progressive muscular atrophy

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7
Q

what type of MND only affects upper motor neurones

A

primary lateral sclerosis

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8
Q

what are upper motor neurone signs

A

increased tone
hyper-reflexia
spastisity
babinksi sign

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9
Q

what are lower motor neurone signs

A

decreased tone
decreased reflexes
fasciculations

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10
Q

what cognitive problem is ALS linked to

A

frontotemporal dementia

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11
Q

difference between bulbar + Pseudobulbar palsy

A

bulbar palsy – LOWER MN lesion affecting cranial nerves 9 - 12
Pseudobulbar palsy - UPPER MN lesion affecting cranial nerves 9- 12

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12
Q

symptoms of bulbar palsy + Pseudobulbar palsy

A

dysarthria, dysphasia, nasal regurgitation

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13
Q

what is exaggerated jaw jerk a sign of

A

UMN bulbar sign

- Pseudobulbar palsy

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14
Q

how does the tongue appear in bulbar palsy

A

weak, wasted + fasciculations

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15
Q

how does the tongue appear in Pseudobulbar palsy

A

small + spastic

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16
Q

most common cause of Pseudobulbar palsy

A

stroke

17
Q

pathological hallmark seen in 95% of MND cases

A

TDP 43

18
Q

how is diagnosis of MND made

A

based on clinical picture + exclusion of other causes

  • UMN + LMN signs in absence of sensory symptoms
  • MRI of brain + spinal cord excluded structural causes
  • LP excludes inflammatory causes
19
Q

pathology of primary lateral sclerosis

A

loss of Betz cells in motor cortex

20
Q

what drug can slow progression of MND

A

riluzole

- sodium channel blocker that decreases glutamate release