Motor Neuron Disease Flashcards Preview

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Flashcards in Motor Neuron Disease Deck (16):
1

Patient presents with her right foot "catching" and rolling her ankle for past few months. Recently, left foot also catching and some twitching in her calves.

ALS is on your differential... (case presented in class)

2

Patient presents with asymmetric, distal weakness and atrophy, fasciculations, and cramps

Motor nerve disease (NOTE: atrophy is super notable in motor nerve disease; cramp is usually due to a whole muscle firing up)

3

Patient presents with weakness that is fluctuating and fatigue-able (i.e. muscles get weaker with use)

Disease of neuromuscular junction

Think:
Myasthenia Gravis: bulbar, ocular involvement common
Lambert-Eaton Myasthenic Syndrom - Usually proximal and symmetric - bulbar involvement rare.

4

Patient presents with weakness that is fluctuating and fatigue-able (i.e. muscles get weaker with use) and bulbar and ocular involvement

Myasthenia Gravis

5

Patient presents with weakness that is fluctuating and fatigue-able (i.e. muscles get weaker with use) bilaterally in her proximal muscles

Lambert-Eaton Myasthenic Syndrom - Usually proximal and symmetric - bulbar involvement rare.

6

Patient presents with proximal, non-fluctuating, symmetric, painless weakness

Muscle disease

7

What symptoms are associated with upper motor neuron dysfunction?

Hyperreflexia,
Babinksi and Hoffman signs
Increased tone/spasticity
Slowness of movement

8

What symptoms are associated with lower motor dysfunction?

Hyporeflexia,
Atrophy,
Fasciculations
Cramps

9

Diminished reflexes suggest

peripheral nervous system problem:
1. Roots/Plexus/Nerves (VERY decreased)
2. Sensory Nerves only (VERY decreased)
3. Motor nerves only (VERY decreased)
4. NMJ (moderately decreased)
5. Muscle (moderately decreased)

10

Name two diseases of upper motor neurons ONLY

1. Hereditary Spastic Paraparesis (HSP)
2. Primary Lateral Sclerosis (PLS)

11

Name one disease that involves both upper and lower motor neurons.

Amyotrophic Lateral Sclerosis (ALS)

NOTE: It could be confused with a spinal cord compression, but in that case you would expect some sensory complaints

12

What kinds of viruses can cause lower motor neuron disease?

Polio, polio-like enteroviruses, West Nile

13

What are some non-motor manifestations of ALS?

Weight loss, fatigue, pain due to immobility, pseudobulbar affect (emotional lability - so they might cry or laugh really easily, without corresponding changes in how the patient feels), depression affects 10%, cognitive changes resembling FTD in 5% (specifically changes in behavior, mood, and executive function + language problems)

(note that sensory signs are very unusual despite dropout of DRG cells at autopsy and loss of small fibers in skin biopsy)

14

What do you call clinically evident muscle atrophy?

Amyotrophy

15

What percent of ALS is familial?

10

16

How might you distinguish between UMN and LMN dysarthria?

Central (UMN) dysarthria: "spastic dysarthria" - speed is slow (and rhythm is a little off), but articulation is fine

Peripheral (LMN) dysarthria: "nasal dysarthria" - speed (and rhythm) is normal, articulation is poor; palatal weakness --> nasal quality