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Conditions neuro > Degenerative Diseases and Disorders of the Motor Unit > Flashcards

Degenerative Diseases and Disorders of the Motor Unit Flashcards

1
Q

What is Amyotrophic Lateral Sclerosis (ALS)?

A

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.

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2
Q

What are the two forms of ALS?

A

Sporadic (SALS) and Familial (FALS)

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3
Q

What are some possible theories of the etiology of ALS?

A

Metabolic disorders, glutamate insufficiency, metal toxicity, autoimmune factors, genetic factors, and viral infection.

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4
Q

What is the clinical picture of the end stages in ALS? (In terms of eating and breathing)

A

The use of tube feedings and a ventilator

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5
Q

What does ALS not affect?

A

Cognition, eye function, sensory, or B &B function

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6
Q

What is stage 1 of ALS?

A

Mild weakness, clumsiness, ambulatory, independence in ADLs

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7
Q

What is stage 2 of ALS?

A

Moderate selective weakness

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8
Q

What is stage 3 of ALS?

A

Severe selective weakness: ankles, wrists, hands.

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9
Q

What is stage 4 of ALS?

A

Severe LE weakness, w/c dependent, hanging arm syndrome.

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10
Q

What is stage 5 of ALS?

A

Severe LE weakness, w/c dependent, moderate to severe UE weakness.

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11
Q

What is stage 6 of ALS?

A

Dependent

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12
Q

What is Huntington’s Disease?

A

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.

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13
Q

What is the etiology of HD?

A

Triggering mechanism for the neuronal degeneration is unknown, but it is linked to coding on the chromosome 4.

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14
Q

What are some symptoms of HD?

A
  • Progressive disorder of both voluntary and involuntary movement.
  • Deterioration of motor control, cognitive and emotional function.
  • Chorea
  • Depression
  • Dysarthria, Dysphasia
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15
Q

What is Parkinson’s Disease?

A
  • 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.
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16
Q

What are some things that you would see in the clinical picture of PD?

A
  • cogwheel rigidity
  • B & B incontinence
  • Resting tremor
  • Mask-like face
  • Micrographia
  • Muscle rigidity
17
Q

What are some OT interventions for PD?

A
  • Compensatory strategies
  • Client/family education and training
  • Environmental and task modification (adapt/grade)
  • AE
  • Address dysphagia/dysarthria
  • PNF/exercise/rhythmic stimulation
  • Community support/resources
18
Q

What is multiple sclerosis (MS)?

A

Progressive neurological disease that damages the myelin sheath in the CNS.

19
Q

What is the typical onset of MS?

A

Between the ages of 20 and 45

20
Q

What is the pathophysiology of MS?

A

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.

21
Q

What are the different disease course classifications of MS?

A
  1. Relapsing-remitting
  2. Secondary-progressive
  3. Primary-progressive
  4. Progressive-relapsing
22
Q

What is relapsing-remitting MS?

A

It is clearly defined acute attacks with either full recovery, or with residual deficit upon recovery.

23
Q

What is secondary-progressive MS?

A

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.

24
Q

What is primary-progressive MS?

A

It is a progression of disability from onset (downward course) without plateaus or remissions, or with occasional plateaus and temporary minor improvements.

25
Q

What is progressive-relapsing MS?

A

It is the least common disease course. Shows progression from onset but with clear acute relapses with or without full recovery.

26
Q

What are some symptoms of MS?

A
  • fatigue
  • ambulation
  • B & B
  • visual disturbances
  • cognitive problems
  • tremor
  • movement problems
27
Q

What are some OT interventions for MS?

A
  • 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
28
Q

What are some OT interventions for HD?

A

Dependent on stage of disease. However interventions should address:

  • cognitive components
  • work site evaluation
  • psychosocial issues
  • social context
  • motor disturbances
  • family training

Conditions neuro (11 decks)

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