Motor Neuron Disease - Sachen Flashcards Preview

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Flashcards in Motor Neuron Disease - Sachen Deck (22)
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1

Motor neuron disease is characterized by what symptoms?

Do NOT see what symptoms?

- Progressive wasting
- Weakness


- Sensory changes
- Cerebellar changes
- Mental changes

2

Polio - causes what?

PATCHY destruction of various anterior horn cells

3

Acid maltase deficiency

Destruction of anterior horn cells

4

Most common adult motor neuron disease?

Affects what cells?

Amyotrophic lateral sclerosis

Pyramidal cells (UMNs)
***Anterior horn (LMNs)
Lower cranial nerve nuclei
***Corticospinal tracts (Lateral, Ventral)
Corticobulbar tracts

5

Progressive bulbar palsy -- affects what cells?

Lower cranial nerve nuclei and associated nerves

6

Spinal muscular atrophy -- affects what cells?

Anterior horn cells

7

Primary lateral sclerosis -- affects what cells?

Corticospinal tracts

8

Monomelic amyotrophy -- what is it?

2 types

Muscle weakness and atrophy of only ONE limb

- Upper extremity (Hirayama)
- Lower extremity

9

How are adult motor neuron diseases distinguished? (2)

- Distribution (limb and/or bulbar)
- Upper and/or lower motor neuron

10

What does "Amyotrophic Lateral Sclerosis" mean?

Amyotrophic = without muscle nourishment (innervation)

Lateral = side of cord

Sclerosis = hardening/scaring

11

ALS - age of onset?

Sporadic or familial?

20-60 (usually young adult)

Sporadic (95%)

12

ALS - presentation

- Gait disorder, limb weakness, speech/swallowing difficulty
- Weight loss (lost muscle), cramps, fasciculations
- Tongue atrophy and fasciculations

13

ALS - neuro exam findings

Mixed upper (spasticity, hyperreflexia, Babinski) and lower (atrophy, fasciculations) neuron signs

14

Whenever you see ___ and ___ motor neuron signs in a limb, think ALS

Upper AND Lower

15

5 "Rule of Thumb" Diagnostic NEGATIVES for ALS

- No sensory symptoms
- Normal mentation/thinking
- No EOM issues
- No autonomic issues
- No decubiti/ulcers

16

Progression and Prognosis of ALS

- Relentlessly progressive w/o remissions
- Death (w/in 4 yrs) -- respiratory failure, aspiration pneumonia, PE

17

ALS - treatment

Supportive for symptoms

18

Progressive bulbar palsy - presenting symptoms (4)



Progression?

- Dysarthria
- Dysphagia
- Chewing issues
- Respiratory difficulty

ALS (generalized involvement)

19

Spinal Muscular Atrophy - presentation? (3)

Does NOT include what?

Presents when?

Progression?

- Weakness
- Atrophy
- Respiratory difficulty

NO UMN involvement

64 (average)

Rarely to ALS - better life expectancy

20

Primary lateral sclerosis - presentation? (4)

Affects mostly what?

Presents when?

Progression?

- Weakness
- Spasticity
- Hyperreflexia
- Babinski

UMN (corticospinal tracts)

50-55

21

Hypotonic baby, poor reflexes, poor suckling, breathing difficulty, death in 6-12 months

Infantile SMA (Werdnig-Hoffman disease)

22

Adolescent/young adult, hypotonia, poor reflexes, breathing difficulty, atrophy

Juvenile SMA (Kugelberg-Welander disease)