Motor Neuron Diseases Flashcards
(44 cards)
What is the most common adult onset motor neuron disease?
ALS
Does ALS affect UMN or LMN?
BOTH - it affects both UMN and LMN
Who is most often affected by ALS? Age of onset?
- White males and non-hispanics
- Typical age of onset is 55-75
What is the initial presentation of ALS?
- Asymmetrical, focal, distal weakness of one limb
* *Most commonly the UE > LE > bulbar
What does the progression of ALS look like?
It has a “contiguous progression” meaning it spreads to anatomically adjacent regions
**RUE»_space; LUE; RLE»_space; LLE
What is the clinical presentation of ALS?
- UMN signs (spasticity, clonus, hyper reflexia, and pathological reflexes)
- LMN signs (muscle weakness, muscle atrophy, fasciculations, hypo reflexia, hypotonicity)
- Other symptoms: sialorrhea, dysarthria, dysphagia, pseudobulbar affect, chronic fatigue, difficulties breathing, urinary changes
What functions and motor neurons are typically NOT affected by ALS
- Sensory
- Bowel and bladder
- Cognition
- Sexual function
- Cardiovascular
- Oculomotor
What is the life expectancy of ALS?
Average is 3 years from onset
**20% survive 5 years, 10% survive 10 years, and 5% survive 20 years
Better vs worse prognostic factors for ALS?
- Better = younger onset and having primary lateral sclerosis or spinal muscle atrophy vs classic ALS
- Worse = initial symptoms involving the bulbar muscles or respiratory system
What is the cause of ALS?
UNKNOWN, but it is associated with several genetic mutations
**90% is sporadic and 10% is hereditary, but no difference in clinical presentation
What criteria is used to Dx ALS?
- Presence of UMN signs, LMNS, and spread of sx within a region or to other regions
- Absence of other disease processes - electrodiagnostic testing
What do you expect to see on electrodiagnostic testing with ALS?
- EMG: Reduction in the number of motor units, fibrillations and fasciculations.
- Nerve conduction tests: Usually normal but might be slightly slowed
What medication is use to treat ALS? How does it work? Pros and cons?
Riluzole: It decreases the release of glutamate.
- It increases life expectancy by 2-9 months - It’s very expensive
Exercise intensity recommendations for people with ALS
- High intensity exercise is CONTRAINDICATED in all stages of ALS
- Moderate intensity exercise may be beneficial in EARLY stages only
Use of AD and DME for persons with ALS
- Manual w/c is often not recommended/ordered due to progressive nature of disease
- Power w/c is order early and can be adapted
What causes polio?
Virus poliomyelitis that causes inflammation to the meninges and anterior horn cell with loss of spinal and bulbar motor neurons
What is the clinical presentation of polio?
Asymmetrical, flaccid paralysis, usually LE > UE
What is post polio syndrome?
New onset of symptoms occurring 15-40 years post infection
What are symptoms of post polio syndrome?
- Fatigue
- New respiratory difficulties
- Cold intolerance
- Weakness
- Joint and muscle pain
- Decreased endurance
What is the progression like in post polio syndrome?
Slow and steady progression: 1-2% increase in weakness per year
Why do they think post polio syndrome occurs?
Failure of the oversized motor units that developed due to collateral sprouting
What factors increase the likelihood of developing post polio syndrome?
- > 10 years old
- Requiring hospitalization for the illness
- Paralytic involvement of all four limbs
- Vent dependent
- Rapid return of function following extensive involvement
Physical therapy management of persons with post polio syndrome (interventions, focus of treatment, etc)
- Energy conservation technique, submaximal short duration strength training, cardiovascular conditioning, stretching, decrease stress on joints, respiratory techniques,
- Education: Fatigue and over work weakness are common, need to educate on submax exercise and energy conservation
How many people who had polio will develop post polio syndrome?
25-40%
