Degenerative Diseases and Disorders of the Motor Unit Flashcards
What is Amyotrophic Lateral Sclerosis (ALS)?
A group of progressive and degenerative neuromuscular diseases characterized by the destruction of motor neurons (UMN & LMN) within the spinal cord, brain stem, and motor cortex.
What are the two forms of ALS?
Sporadic (SALS) and Familial (FALS)
What are some possible theories of the etiology of ALS?
Metabolic disorders, glutamate insufficiency, metal toxicity, autoimmune factors, genetic factors, and viral infection.
What is the clinical picture of the end stages in ALS? (In terms of eating and breathing)
The use of tube feedings and a ventilator
What does ALS not affect?
Cognition, eye function, sensory, or B &B function
What is stage 1 of ALS?
Mild weakness, clumsiness, ambulatory, independence in ADLs
What is stage 2 of ALS?
Moderate selective weakness
What is stage 3 of ALS?
Severe selective weakness: ankles, wrists, hands.
What is stage 4 of ALS?
Severe LE weakness, w/c dependent, hanging arm syndrome.
What is stage 5 of ALS?
Severe LE weakness, w/c dependent, moderate to severe UE weakness.
What is stage 6 of ALS?
Dependent
What is Huntington’s Disease?
A fatal, degenerative neurological disorder that is transmitted in an autosomal dominant pattern. It is caused by a mutation on chromosome 4. Each offspring of an affected parent has a 50% of having HD.
What is the etiology of HD?
Triggering mechanism for the neuronal degeneration is unknown, but it is linked to coding on the chromosome 4.
What are some symptoms of HD?
- Progressive disorder of both voluntary and involuntary movement.
- Deterioration of motor control, cognitive and emotional function.
- Chorea
- Depression
- Dysarthria, Dysphasia
What is Parkinson’s Disease?
- It is a degeneration of neurons in the substantia nigra which produces a decrease in activity within the basal ganglia and an overall reduction in spontaneous movement.
- Slow progressive, degenerative movement disorder. It isn’t considered fatal, but PD severely compromises functional performance. The Dx is usually made after age 55.
What are some things that you would see in the clinical picture of PD?
- cogwheel rigidity
- B & B incontinence
- Resting tremor
- Mask-like face
- Micrographia
- Muscle rigidity
What are some OT interventions for PD?
- Compensatory strategies
- Client/family education and training
- Environmental and task modification (adapt/grade)
- AE
- Address dysphagia/dysarthria
- PNF/exercise/rhythmic stimulation
- Community support/resources
What is multiple sclerosis (MS)?
Progressive neurological disease that damages the myelin sheath in the CNS.
What is the typical onset of MS?
Between the ages of 20 and 45
What is the pathophysiology of MS?
White blood cells attack myelin and nerve fibers, causing inflammation and destruction and thus obstructing nerve signals. MS lesions show up as hardened scars/plaques.
What are the different disease course classifications of MS?
- Relapsing-remitting
- Secondary-progressive
- Primary-progressive
- Progressive-relapsing
What is relapsing-remitting MS?
It is clearly defined acute attacks with either full recovery, or with residual deficit upon recovery.
What is secondary-progressive MS?
It begins with an initial relapsing-remitting disease course, followed by progression form. It has a variable rate that may include occasional relapses and minor remissions and plateaus.
What is primary-progressive MS?
It is a progression of disability from onset (downward course) without plateaus or remissions, or with occasional plateaus and temporary minor improvements.
What is progressive-relapsing MS?
It is the least common disease course. Shows progression from onset but with clear acute relapses with or without full recovery.
What are some symptoms of MS?
- fatigue
- ambulation
- B & B
- visual disturbances
- cognitive problems
- tremor
- movement problems
What are some OT interventions for MS?
- Disease education
- Dx of emotional/cognitive problems
- Family intervention/training
- Support groups
- Resources
- UE strengthening
- Movement/coordination
- Assistive Technology
- Fatigue management through EC & WS
- Compensatory strategies
- Stress management
What are some OT interventions for HD?
Dependent on stage of disease. However interventions should address:
- cognitive components
- work site evaluation
- psychosocial issues
- social context
- motor disturbances
- family training
