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.
What is the most common adult onset motor neuron disease?
ALS
What functions and motor neurons are typically NOT affected by ALS
typically the following are spared: sensory, bowel and bladder, cognition, sexual function, cardiovascular, oculomotor
Axonotmesis: class/grades
Class 2. Grade 2 = axonal damage. Full recovery. Grade 3 = axonal and endoneurium. Varied recovery. Grade 4= axonal, endoneurium, and perineurium, no recovery expected, needs surgery
medical management of GBS- what is recommended? What is NOT recommended?
- ) (IVIg) intravenous immunoglobulin therapy is recommended over plasma exchange therapy because its easier to administer and is associated with fewer complications.
- ) Corticosteriods have not shown to be affective. Actually delays recovery
Does ALS affect UMN or LMN?
Both. It affects both UMN and LMN
Neurotmesis
Class 3 and Grade 5: complete transection of nerve including epineurium. No recovery, Needs surgery
what type of disease is GBS
an autoimmune disorder of the PNS
what is the clinical presentation of ALS?
you will see UMN signs (spasticity, clonus, hyper reflexia, and pathological reflexes) and LMN signs (muscle weakness, muscle atrophy, fasciculations, hypo reflexia, hypotonicity). Other symptoms: sialorrhea, dysarthria, dysphagia, pseudobulbar affect, chronic fatigue, difficulties breathing, urinary changes.
What is the cause of ALS?
the cause is unknown, but it is associated with several genetic mutations. 90% is sporadic and 10% is hereditary, but no difference in clinical presentation
what is the medical management for myasthenia gravis?
corticosteroids or immunosuppressants
what is the initial presentation of ALS?
ALS initially presents as asymmetrical, focal, distal weakness of one limb. Most commonly the UE > LE > bulbar
What criteria is used to Dx ALS?
The world Federation of Neurology El Escorial Criteria. Presence of UMN signs, LMNS, and spread of sx within a region or to other regions. Absence of other disease processes. Use of electrodiagnostic testing
what are the two systems for classifying peripheral nerve damage?
seddon (class 1-2) and sunderland (Grades 1-5)
what causes polio?
polio is caused by the virus poliomyelitis that causes inflammation to the meninges and anterior horn cell with loss of spinal and bulbar motor neurons
What causes GBS?
- following non specific viral syndrome (IE mild upper respiratiory infection)
- also associated with viral infections, Campylobacter jejuni bacterial infection, vaccinations or recent surgery, lymphoma, systemic lupus, AIDS
who is most often affected by ASL? Age of onset?
mostly affects white males and non-hispanics. Typical age of onset is 55-75 but can occur form late teens to very old
what are symptoms of post polio syndrome
fatigue, new respiratory difficulties, cold intolerance, weakness, joint and muscle pain, decreased endurance
who does myasthenia gravis usually affect?
younger females and older males
what are some trophic changes that can occur with peripheral neuropathy
shiny skin and brittle nails
how fast/slow does GBS progress?
onset to peak disability ranges from hours to week
How has peripheral neuropathy affect muscles?
causes weakness due to denervation of muscles and disuse atrophy
types of acquired peripheral nerve neuropathy
- Trauma 2. systemic disease 3. infection/autoimmune.
- ) Trauma (traction, laceration, compression, repetition), 2.) Systemic disease (DM, vit decencies, alcohol, vascular damage, cancer/tumor, hormonal imbalances, toxins) 3.) Infections / autoimmune disorders (HIV, Lyme disease, viral/bacterial infections, CIDP)
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 is the hallmark sign of Myasthenia Gravis?
fluctuating fatigue and strength. Weakness/fatigue increase during activity and improves with rest. Environmental factors can also affect symptoms
