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CMT > Degenerative Motor Neuron Diseases > Flashcards

Degenerative Motor Neuron Diseases Flashcards

1
Q

characterized by

A

weakness and wasting of muscles without sensory changes

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2
Q

Examples

5

A
  1. bulbar palsy
  2. pseudobulbar palsy
  3. progressive spinal muscular atrophy
  4. primary lateral sclerosis
  5. amyotrophic lateral sclerosis
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3
Q

common sx of all disorders

6

A
  1. dysphagia
  2. problems w/ chewing
  3. problems w/ coughing
  4. dyspnea
  5. trouble w/ talking
  6. limb weakness
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4
Q

general tx options

A
  • edaravone (60mg infusion for 10-14d monthly)
  • slows disease progression in mild cases
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5
Q

other general tx options

5

A
  • botox injections
  • lots of PT/OT
  • suction machines
  • feeding tube
  • tracheostomy
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6
Q

Bulbar Palsy

primarily affects what?

A

motor nuclei of cranial nerves

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7
Q

Bulbar Palsy

hallmark sx

2

A
  1. drooping of palate
  2. weak tongue
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8
Q

Bulbar Palsy

worry with this?

all the sx combined can lead to…..

A

depressed gag reflex + weak cough + pooling of saliva in pharynx = aspiration

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9
Q

Pseudobulbar Palsy

dysfunction with what

A

upper motor neurons

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10
Q

Pseudobulbar Palsy

hallmark sx

4

A
  1. uncontrolled episodes of laughing or crying without cause
  2. tongue is contracted + spastic, difficult to move
  3. stiff and weak limbs, muscle wasting, fasciculations
  4. sphincters are spared
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11
Q

differentiate bulbar palsy and pseudobulbar palsy

A
  • Gag reflex: bulbar absent, pseudo normal
  • jaw jerk: bulbar absent, psuedo normal
  • tongue: bulbar wasted w/ fasciculation, pseudo spastic w/out fasciculation
  • emotional: bulbar emotional liability, pseudo no emotional liability
  • speech: bulbar nasal, pseudo hot potato?
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12
Q

Progressive Spinal Muscular Atrophy

describe

A

lower motor neuron deficit in limbs due to degeneration of anterior horn cells of spinal cord

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13
Q

Primary Lateral Sclerosis

describe

A
  • upper motor neuron deficits only in the limbs
  • typically have longer life but profound quadriparesis and spasticity
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14
Q

Amyotrophic Lateral Sclerosis

describe location of deficits

A
  • mixed upper and lower motor neuron deficit but no sensory deficits
  • affects frontal motor neurons w/ UMN degeneration and LMN degeneration
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15
Q

Amyotrophic Lateral Sclerosis

sx of UMN degeneration

3

A
  • weakness
  • hyperreflexia
  • spasticity
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16
Q

Amyotrophic Lateral Sclerosis

sx of LMN degeneration

3

A
  • weakness
  • atrophy
  • fasciculation
17
Q

Amyotrophic Lateral Sclerosis

describe onset

A

gradual, over a period of time over several months w/ progressive worsening

18
Q

Amyotrophic Lateral Sclerosis

what is required for official dx

A

changes in all 3 spinal regions

19
Q

Amyotrophic Lateral Sclerosis

PE Findings

9 components

A
  1. clumsiness, gait abnormalities, limb weakness, wrist/foot drop, poor fine motor skills
  2. muscle fasciculations
  3. voice changes
  4. involuntary laughing/crying
  5. drooling
  6. dyspnea on exertion
  7. cognitive changes
  8. sesnation in tact
  9. hyperreflexia + pronator drift
20
Q

Amyotrophic Lateral Sclerosis

dx

3 components

A
  • EMG to r/o other causes
  • labs to r/o other causes
  • no specific test to dx ALS, dx based on H&P
21
Q

Amyotrophic Lateral Sclerosis

tx

A
  1. focused on survival and symptom management (respiratory function)
  2. use orthotic and assistive devices to keep pt mobile
  3. physical, occupational, speech therapies
22
Q

Amyotrophic Lateral Sclerosis

meds which can help slow progression

3 components

A
  • riluzole (50mg PO QD)
  • edaravone (60mg IV QD for 2 wks on and off; then 10 days on, 2 wks off)
  • muscle relaxants and botox injections for spasticity
23
Q

Amyotrophic Lateral Sclerosis

usually fatal within?

A

3-5 yrs because of pulm infections

24
Q

Amyotrophic Lateral Sclerosis

survival rate 5-10 yrs post onset

A

20%

CMT (72 decks)

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