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Neuro > ALS > Flashcards

ALS Flashcards

1
Q

describe epidemiology of ALS

A

onset of mid to late 50s.
5-10% have family hx

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2
Q

what percentage of ALS cases are limb-onset and what percent are bulbar-onset?

A

70-80% limb
20-30% bulbar

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3
Q

describe the etiology of ALS

A

most cases have unknown etiology but small percent are hereditary.

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4
Q

what is the hallmark sign of ALS?

A

UMN and LMN pathology

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5
Q

what cranial nerves are involved with ALS?

A

5,7,9,10,12

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6
Q

ALS involves a degeneration of motor neurons in 4 areas…what are they?

A

primary motor cortex, corticospinal tract, brainstem, spinal cord

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7
Q

can’t neighboring neurons help out the neurons that are denervated?

A

yes but only for a certain amount of time. Once more than 50% of motor units are lost they can’t help anymore

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8
Q

talk about the progression of ALS throughout the body

A

it spreads throughout a whole region before moving onto a different region. It also begins distal then moves proximal.

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9
Q

what are the most frequent clinical manifestations of ALS?

A

focal asymmetrical muscle weakness that begins in either UE or LE. Weakness of the bulbar muscles.

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10
Q

what are examples of LMN sxs?

A

muscle weakness, hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations

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11
Q

what are examples of UMN sxs?

A

spasticity, babinski, hoffman, hyperreflexia, muscle weakness

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12
Q

what are the impairments associated with Bublar pathology?

A

facial and neck weakness, dysphagia, dysarthria, sialorrhea, pseudobulbar affect

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13
Q

what is the definition of cachexia?

A

extreme weight loss and wasting

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14
Q

how often are cognitive impairments in ALS and what type of cognitive impairment is associated with ALS?

A

35% of people with ALS. Frontotemporal dementia

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15
Q

what is pseudobulbar affect?

A

exaggerated crying and then laughing, untriggered

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16
Q

why is fatigue common in ALS?

A

because the surviving motor units are overburdened.

17
Q

what ocular pathology is related to ALS?

A

none really. if the pt lives longer then they may have opthalmoplegia

18
Q

how are the sphincter muscles affected in ALS?

A

generally spared

19
Q

what must a pt have in order for an ALS diagnosis?

A

LMN signs by clinical exam, EMG, or neuro examination
UMN signs clinical exam
Progression of the disease within a region or other regions by clinical exam

20
Q

according to the el escorial criteria, what must a pt have in order to have definite ALS?

A

LMN and UMN signs in 3 regions

21
Q

according to the el escorial criteria, what must a pt have in order to have definite Familial ALS?

A

LMN and UMN sxs in 1 region and identified DNA gene

22
Q

according to El Escorial criteria, if a pt has LMN sxs in 2 regions but no UMN sxs, what type of ALS diagnosis would they have?

A

suspected ALS

23
Q

what is the average duration for onset to death?

A

27-43 months

24
Q

what typically causes death in ALS patients?

A

respiratory failure

25
Q

describe the different factors for prognosis in ALS

A

Age is a strong predictor. onset less than 35-40.
Type of ALS. Limb onset is less aggressive than bulbar.
Psychological distress increase risk of death.
Nutrition. Many presents with dehydration and malnutrition

26
Q

what are some referrals you could make for an ALS pt?

A

OT, SLP, nutrition, pharmacist, RT, home health, psychologist, social worker, chaplain, neurologist

27
Q

what medication has shown to extend ALS survival rate by 2-3 months?

A

Riluzole

28
Q

When is ventilation recommended for ALS pts?

A

when VC is below 50%

29
Q

when is psychological support recommended in the ALS disease stage? (early, middle, late)

A

the whole time

30
Q

what stage of ALS is associated with min/mod activity limitations? (early, middle, late)

A

Middle

31
Q

What stage of ALS does the intervention strategy become compensation and prevention?

A

Middle

32
Q

what are some example outcome measures for postural control/balance?

A

POMA, BERG, TUG, FR

33
Q

what stage of ALS does the pt commonly require a WC for long distances?

A

middle

34
Q

when are soft foam collars worn vs semi-rigid/rigid collars worn?

A

soft foam collars are worn for mild to moderate weakness. rigid collars are worn for moderate to severe weakness.

35
Q

what must one consider when prescribing KAFOs to an ALS pt?

A

how much they weigh. this will cause fatigue.

36
Q

on the MMT scale, what rating does a muscle have to be to not suffer from overuse weakness?

A

greater than or equal to 3/5

37
Q

according to the CPG for pts with ALS, exercise programs should include what 4 things?

A

maintaining ROM
Optimizing fx
Preventing contractures
Reducing pain

38
Q

what are some important things to educate pts with ALS on so that they safely exercise?

A

Moderate resistance
Use fatigue as guide for aerobic activity
Limit eccentric contractions

Neuro (3 decks)

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