Clin: Motor Neuron Disease Flashcards
What type of signs/sx’s seen with ALS?
- Mixed upper (spasticity, hyperreflexia, Babinski sign)
and
- LMN (atrophy, fasciculations) signs
What will an EMG of pt with ALS show?
Widespread denervation** and **reinnervation
What are the sensory sx’s seen with ALS?
NONE
The pathophysiology of ALS is due to degeneration of which 4 things?
- Betz cell
- Lower brainstem nuclei
- Descending corticospinal tracts
- Anterior horn cells
Progressive Bulbar Palsy is due to selective involvement of what?
Motor nuclei of the lower CN’s
What are some of the signs/sx’s of Progressive Bulbar Palsy; describ the course of the disease?
- Dysarthria + dysphagia + dysphonia + chewing difficulty + drooling
- Almost always progresses to generalized disease i.e., ALS
Which deficits predominate in (progressive) spinal muscular atrophy; mean age of onset?
- Mean age = 64 y/o
- LMN deficits predominate due to degeneration of anterior horn cell
- NO upper motor neuron involvement
Typical presenting sx’s of (progressive) spinal muscular atrophy?
- Often begins with symmetric upper extremity involvement
- Weakness + atrophy + respiratory difficulty
What is the prognosis of (progressive) spinal muscular atrophy?
Survival rate ≥15 years (better w/ earlier age of onset)
Which deficits prevail in Primary Lateral Sclerosis; what are the signs/sx’s?
- Upper motor neuron (corticospinal) deficits prevail
- Weakness, spasticity, hyperreflexia, Babinski signs
What is the progression and survival rate like for Primary Lateral Sclerosis?
- Slow progression, but can evolve into ALS
- Survival rate better than ALS
