Lecture 4: Motor Neuron Disease

Question 1

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

Answer 1

• Mixed upper (spasticity, hyperreflexia, Babinski sign)

and

• LMN (atrophy, fasciculations) signs

Question 2

What will an EMG of pt with ALS show?

Answer 2

Widespread denervation** and **reinnervation

Question 3

What are the sensory sx’s seen with ALS?

Answer 3

NONE

Question 4

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

Answer 4

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

Question 5

Progressive Bulbar Palsy is due to selective involvement of what?

Answer 5

Motor nuclei of the lower CN’s

Question 6

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

Answer 6

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

Question 7

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

Answer 7

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

Question 8

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

Answer 8

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

Question 9

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

Answer 9

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

Question 10

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

Answer 10

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

Question 11

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

Answer 11

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