Motor Neuron Diseases Flashcards
how many people who had polio will develop post polio syndrome?
25-40%
What is the most mild type of peripheral nerve damage? what class/grade is that?
Neuropraxia (class 1 and grade 1): local conduction block or demyelination. Small part of the nerve. Complete and fast recovery is expected
What does Myasthenia Gravis affect?
- Oculomotor
- Facial
- Mastication
- Speech and swallowing muscles
what is post polio syndrome
new onset of symptoms occurring 15-40 years post infection
what is the clinical presentation of polio?
asymmetrical, flaccid paralysis, usually LE > UE
what is the life expectancy of ALS?
the average is 3 years from onset. 20% survive 5 years, 10% survive 10 years, and 5% survive 20 years
who is most affected by GBS?
men > women
ages 30-50 yr old
what are some autonomic symptoms that can occur with peripheral neuropathy
- abnormal sweating
- changes in BP regulation (OH)
- abnormal HR
- bowel and bladder changes
What are the layers of connective tissue surrounding the peripheral spinal nerve?
-outer layer - in (E-P-E): epineurium, perineurium, endoneurium.
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
why do they think post polio syndrome occurs?
theorized that it is due to failure of the oversized motor units that developed due to collateral sprouting
Does GBS affect UMN or LMN?
LMN. CNS is unaffected.
what is the mechanism of GBS?
it is an autoimmune disorder of the PNS. the inflammatory process affects the schwann cells causing de-myelination of the axon. Axon can remain intact or in some cases axonal damage occurs
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
Exercise intensity recommendations for people with ALS
high intensity exercise is contra-indicated in all stages of ALS. Moderate intensity exercise may be beneficial in EARLY stages only.
use of AD and DME for persons with ADL
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 is the clinical presentation of GBS?
- Progressive symmetrical weakness of the limbs and face. Legs to arm, to face
- Paresthesias in the hands and feet in a glove and stocking distribution
- Back pain
- Diminished/absent DTR
- CN are affected in ~50% of all cases
What does the progression of ALS look like?
it has a “contiguous Progression” meaning it spreads to anatomically adjacent regions
what is the progression like in post polio syndrome?
slow and steady progression: 1-2% increase in weakness per year
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
What medication is use to treat ALS? how does it work? pros and cons?
Riluzole is the only drug approved by the FDA to treat ALS. It decreases the release of glutamate. It increases life expectancy by 2-9 months. It’s very expensive
what are the rehab implications for people with myasthenia gravis?
- energy conservation
- risk for falls with decreased bone density
PT/exercise considerations for patients with GBS?
- Watch for overwork weakness due to DOMS 1-5 days post activity and Reduction in maximum force production that gradually recovers
- Train fast twitch muscle contractions
- Monitor vitals during aerobic exercise due to autonomic dysfunction
type of inherited peripheral neuropathy and symptoms?
Charcot marie tooth disease - associated with leg weakness and foot deformities. slow progressive condition affecting motor and sensory nerves. Onset is adolescence to mid adult.
