ALS and Gullian Barre Syndrome Flashcards

1
Q

Amyotrophic Lateral Sclerosis (ALS) is aka as _ _ disease, is a degenerative disease that affects both _ and _ and is the _ _ type of _ _ disease.

A

Is aka LOU GEHRIG’S DISEASE, is a degenerative disease that affects both UMN’s and LMN’s and is the MOST COMMON type of MOTOR NEURON DISEASE.

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2
Q

The alsa.org is a great resource for _ and _ _ for patients and caregivers.

A

INFORMATION and MEDICAL EQUIPMENT

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3
Q

Sporadic ALS accounts for %-% of cases and familial ALS accounts for -%. Affects _ more than _. Ratio? Evens out after?

A

Sporadic accounts for 90-95% of cases and familial ALS accounts for 5-10%.

Affects MEN more than WOMEN. Ratio of 1.7:1

Evens out after age 65

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4
Q

Age on onset is typically - for ALS. Mortality is typically - years, however it can be quicker. Disease usually progresses _ within a _ _.

A

Age of onset is typically 50-60

Mortality is typically 3-5 years from onset, however it can be quicker

Disease usually progresses RAPIDLY with a FEW MONTHS

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5
Q

ALS is hypothesized to occur as the result of an intersection of a _ of _ _, leading to _ degeneration and _. Research suggests an excess of _ in the synaptic cleft triggers a _ of _ that leads to _ _.

A

As a result of the intersection of a VARIETY OF CELLULAR INSULTS, leading to NEURON degeneration and DEATH.

Research suggests an excess of GLUTAMATE in the synaptic cleft triggers a CASCADE OF EVENTS that leads to CELLULAR DEATH.

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6
Q

-% develop limb onset with extremities initially involved. -% develop bulbar onset (especially in _ _ _), and is usually noticed first in _, _ or _.

A

70-80% develop limb onset with extremities initially involved.

20-30% develop bulbar onset (especially in MIDDLE AGED WOMEN), and is usually noticed first in SPEAKING, CHEWING, OR SWALLOWING

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7
Q

Pathophysiology: progressive _ _ loss in _ _, _, and _. With partial sparing of CN _, _ and _, _ spinal level, _ tract, _ and _ muscle, _ tracts and _.

A

Progressive MOTOR NEURON loss in SPINAL CORD, BRAINSTEM and CORTEX.

With partial sparing of CN III, IV, and VI, SACRAL spinal level (bowel/ bladder control), SPINOCEREBELLAR tract, SMOOTH and CARDIAC muscle, SENSORY tracts (eyesight), and COGNITION (aware of degeneration).

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8
Q

Diagnostic criteria: Objective evidence of _ and _ damage with a minimum of _ out _ regions affected. Name regions.

A

Objective evidence of UMN and LMN damage with a minimum of 3 out of 4 regions affected.

Regions: CBLT
Cervical, Bulbar (CN X,XI, XII), Lumbosacral, Thoracic

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9
Q

Diagnostic criteria scale is known as _ _ Criteria: clinically _ (/), clinically _ (/), clinically _ with _ support, and clinically _.

A
Scale is known as EL ESCORIAL CRITERIA: 
Clinically DEFINITE (3/4 regions)
Clinically PROBABLE (2/4)
Clinically PROBABLE with LAB support (EMG is slower)
Clinically POSSIBLE
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10
Q

ALS affects every patient _, typically progresses in _ _ first and then _ _ to _ _ _.

A

Affects every patient DIFFERENTLY, typically progresses in ONE AREA first and then MOVES ON to ANOTHER MOTOR AREA.

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11
Q

Rilutek is a medication for ALS, increases survival rate - _ on average.

A

Increases survival rate 2-3 MONTHS on average

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12
Q

3 major stages and associated PT interventions

A

Early Stage- Restorative

Middle Stage: partially independent- Compensatory

Late stage: dependent- Preventative

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13
Q

During the early stage will get some _ due to _ _. In the late stage interventions such as a _ _, _, _ training with _ _, and _ home can be helpful.

A

Early stage will get some FLUCTUATION due to NERVE REGENERATION

In the late stages interventions such as HOYER LIFTS, CUSHIONING, TRANSFER training with SLIDING BOARD, and AUTOMATED home can be helpful.

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14
Q

Cognition, Sensation, Pain, CN integrity, ROM, Muscle performance, tone/ reflexes, motor function, postural alignment, balance, gait/ locomotion, functional status and integumentary system are all important parts of the?

A

Physical examination

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15
Q

During the examination PT may need to _ and/ or _ _ of each over _ _.

A

May need to PRIORITIZE and/ or SPREAD EVALUATION of each over SEVERAL VISITS

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16
Q

What tests are most commonly used for: Cognition? Motor Function? Balance (2)? Gait/ locomotion? Functional status?

A
Cognition- MOCA
Motor Function- Modified Ashworth
Balance- Berg & DGI
Gait/ locomotion- TUG
Functional Status- FIM
17
Q

With ALS Reflexes and Tone can be? (2) Depends on?

A

Can be hyper or hypo (reflexive or tonic)

Depends on area of damage

18
Q

With ALS sensory is usually intact so _ can be used to help patient manage damage to the _ _.

A

So CUSHIONS can be used to help patient manage damage to INTEGUMENTARY SYSTEM

19
Q

What are the 3 disease specific measures used with ALS?

A

ALS Functional Rating Scale (ALSFRS)

Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40)
- self report assessment

Maximum Voluntary Iso metric Contraction (MVIC)
-documents how weakness is progressing (patients do not like to take)

20
Q

What APTA practice pattern is associated with ALS?

A

Pattern 5E

21
Q

Goals of treatment with ALS are _ _ _

A

DEPENDENT ON STAGE

22
Q

Sensory deficits if present are managed with _/ _ modifications to prevent _ _.

A

Are managed with BED/ WHEELCHAIR modifications to prevent PRESSURE SORES

23
Q

Exercise training if greater than 3/5 strength includes: _ and _, _ conditioning and _. If less than 3/5 includes: preventing _ _ or _.

A

Greater than 3/5: STRENTH and CONDITIONING, AEROBIC conditioning and FLEXIBILITY

Less than 3/5 includes: preventing OVERUSE FATIGUE or WEAKNESS (will cause damage and disease to progress more quickly)

24
Q

Management for Spasticity includes: _ and minimizing _.

A

ROM and minimizing CONTRACTURES

25
Q

Additional interventions in the earlier stages includes: , _ and _ training. Later interventions may include // _ _.

A

Earlier stages includes: BALANCE, MOBILITY and GAIT training

Later interventions may include SPLINTING/ BRACING/ DURABLE MEDICAL EQUIPMENT (wheelchairs-power or manual, communication devices)

26
Q

Main goal with interventions for ALS patient is _ _ as long as _.

A

PRESERVE FUNCTION as long as POSSIBLE

27
Q

_ _ _ also referred to as _ _ _ _ and _ _ _ is an inflammatory disorder of the peripheral nerves

A

Guillain-Barre Syndrome, AKA Acute inflammatory demylinating polyneuropathy (AIDP) and Landry’s ascending paralysis is an . . .

28
Q

Genetic, gender and age of onset? Mortality?

A

None has been determined

Mortality is about 5%- usually those that don’t seek treatment

29
Q

GBS is an _ _ and 50% of all cases occur shortly after a _ or _ _ such as a _ _ or _.

A

Is an AUTOIMMUNE DISORDER and 50% of all cases occur shortly after a VIRAL or BACTERIAL INFECTION such as a SORE THROAT OR DIARRHEA

30
Q

GBS is due to _ infiltration of _ _ and _ _, followed by - _ _ of myelin that _ _ electrical impulse propagation leading to _ _.

A

Due to LYMPHOCYTIC infiltration of SPINAL ROOTS and PERIPHERAL NERVES, followed by MACROPHAGE-MEDIATED MULTIFOCAL STRIPPING of myelin that PROGRESSIVELY IMPEDES electrical propagation leading to FLACCID PARALYSIS

31
Q

Diagnostic procedures for GBS include a _ _ test, _ _, and - _ impairments.

A

Include a NERVE CONDUCTION test, LUMBAR PUNCTURE, and STOCKING- AND- GLOVE SENSORY impairments

32
Q

Two forms of medical treatment for GBS include _ and _ _ _ _.

A

PLASMAPHERESIS AND HIGH DOSAGE IV IMMUNOGLOBULINS (IVIG)

33
Q

APTA practice pattern associated with GBS is? Impaired motor function and sensory integrity associated with _ or _ _.

A

Pattern 5G

Impaired motor function and sensory integrity associated with ACUTE OR CHRONIC POLYNEUROPATHIES

34
Q

With SOAP documentation for GBS it is important to capture what they _ and _ _.

A

Capture what the CAN AND CAN’T DO