Clin: Motor Neuron Disease Flashcards

1
Q

What type of signs/sx’s seen with ALS?

A
  • Mixed upper (spasticity, hyperreflexia, Babinski sign)

and

  • LMN (atrophy, fasciculations) signs
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2
Q

What will an EMG of pt with ALS show?

A

Widespread denervation** and **reinnervation

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3
Q

What are the sensory sx’s seen with ALS?

A

NONE

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4
Q

The pathophysiology of ALS is due to degeneration of which 4 things?

A
  • Betz cell
  • Lower brainstem nuclei
  • Descending corticospinal tracts
  • Anterior horn cells
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5
Q

Progressive Bulbar Palsy is due to selective involvement of what?

A

Motor nuclei of the lower CN’s

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6
Q

What are some of the signs/sx’s of Progressive Bulbar Palsy; describ the course of the disease?

A
  • Dysarthria + dysphagia + dysphonia + chewing difficulty + drooling
  • Almost always progresses to generalized disease i.e., ALS
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7
Q

Which deficits predominate in (progressive) spinal muscular atrophy; mean age of onset?

A
  • Mean age = 64 y/o
  • LMN deficits predominate due to degeneration of anterior horn cell
  • NO upper motor neuron involvement
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8
Q

Typical presenting sx’s of (progressive) spinal muscular atrophy?

A
  • Often begins with symmetric upper extremity involvement
  • Weakness + atrophy + respiratory difficulty
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9
Q

What is the prognosis of (progressive) spinal muscular atrophy?

A

Survival rate ≥15 years (better w/ earlier age of onset)

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10
Q

Which deficits prevail in Primary Lateral Sclerosis; what are the signs/sx’s?

A
  • Upper motor neuron (corticospinal) deficits prevail
  • Weakness, spasticity, hyperreflexia, Babinski signs
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11
Q

What is the progression and survival rate like for Primary Lateral Sclerosis?

A
  • Slow progression, but can evolve into ALS
  • Survival rate better than ALS
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