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Year 2: Neuromuscular II > Test 1: ALS > Flashcards

Test 1: ALS Flashcards

1
Q

what is ALS

A

most common adult onset motor neuron disease

greek “amyotropic” = no mm nourishment

lateral = portion of SC

sclerosis = mm hardening

aka lou gherig disease

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2
Q

2 ALS classifications

A

familial: family hx (mostly autosomal dominant)

sporadic = no family hx

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3
Q

onset types of ALS

A

70-80% = limb onset ALS

20-30% = bulbar onset ALS

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4
Q

what neurons are involved with ALS

A

UMN and LMN eventually implicated

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5
Q

fatality/progression of ALS

A

rate of deterioration can vary

individual eventually becomes fully dependent

ALS is always fatal

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6
Q

epidemiology of ALS

A

30000 people in the US

4-10 cases out of 100000

higher incidence in men (1.7:1)

average onset is mid to late 50s

5-10% have familial

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7
Q

etiology of ALS

A

largely unknown

possible causes:
- excess glutamate
- SOD-1 mutation
- inflammation/autoimmune
- growth factor abnormalities
- viral infections
- apoptosis (programmed cell death)

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8
Q

known ALS risk factors

A

age
gender (male)
family hx
clusters (western pacific)
disease causing mutations

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9
Q

possible ALS risk factors

A

diet
vigorous PA
trauma
neurotoxicant exposure
lifestyle
certain occupation characteristics (farmers, military, electric workers)

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10
Q

pathophysiology of ALS

A

loss of motor neurons in motor cortex, SC, and brainstem

early on = reinnervation from healthy axons to denervated mm

with disease progression reinnervation cannot keep up with rate of denervation

typical progression = within body region before moving to neighboring region

common CNs affected = V, VII, IX, X, and XII

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11
Q

non-motor areas affected by ALS

A

ANS
basal ganglia
cerebellum
frontotemporal
oculomotor
sensory system

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12
Q

clinical presentations of ALS depend on

A

extent of motor neuron loss

combo of UMN and LMN loss

non-motor areas affected

pattern of onset

body regions affected

secondary impairments

stage of disease

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13
Q

LMN signs you may see with ALS

A

mm weakness
hyporeflexive
hypotonicity
mm cramps
atrophy
fasciculations

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14
Q

UMN signs you may see with ALS

A

spasticity
hyperreflexia
pathological reflexes
mm weakness

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15
Q

clinical presentation of ALS

A

initial onset usually assymetrical S&S and focal weakness (i.e. foot drop)

cervical extensor weakness

fatigue

pain

secondary impairments in balance, ROM, gait, etc

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16
Q

types of bulbar pathologies

A

spastic bulbar palsy (UMN)

flaccid bulbar palsy (LMN)

mixed palsy is common

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17
Q

clinical presentation of bulbar pathology of ALS

A

bulbar mm weakness

dysphagia

dysarthria

sialorrhea

pseudobulbar affect

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18
Q

respiratory impairments that you may see with ALS

A

respiratory mm weakness
dyspnea
nocturnal respiratory difficulty
dysphasgia
orthopenia
hypoventilation
secretion retention
ineffective cough

**respiratory failure = leading cause of death with ALS

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19
Q

patient complaints common with ALS

A

fatigue
decrease activity level
inability to tolerate supine
HA due to hypoxia
changes in speech

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20
Q

cognitive deficits you may see with ALS

A

decreased language comprehension
impaired memory
difficulty with abstract reasoning

frontotemporal dementia

bulbar onset = more predictive of cognitive impairment than limb onset

21
Q

rare clinical manifestations with ALS

A

sensory
bowel/bladder
occular palsy

22
Q

how to dx ALS

A

no single test; dx by ruling out

tests to rule out may include: EMG, lab studies, neuroimaging, nn conduction test, mm/nn biopsy

symptom onset to dx ranges 8-15 months

El Escorial Criteria and Awaji-Shima Criteria can help aide physicians to dx

23
Q

describe what needs to be present/absent for the el escorial criteria

A

presence of:
1. LMN
2. UMN
3. progressive spread of S&S

absence of:
1. electrophysiological or pathological evidence of other diseases
2. neuroimaging of other disease processes

24
Q

life expectancy of ALS

A

majority = death 3-5 years after symptom onset

10% survive longer than 10 years

25
negative prognostic factors for ALS
bulbar onset executive dementia psychological distress
26
positive predictive factors for ALS
younger age of onset limb onset ALS delay between symptom onset and diagnosis
27
pharmacology management options for ALS and the side effects of each
Riluzole (Rilutek): liver toxicty, N&V, dizziness, asthenia Radicava/Radicut (Edaravone): HA, gait problems, respiratory issues, eczema, and dermatitis Reyvrio (AMX0035): upper resp. tract infection, Na retention, abdominal pain, diarrhea, N&V Qalsody (toferson): joint pain, mm cramps, nn pain, fatigue, HA, blurry vision
28
med management for dysphagia
SLP and nutritionish should be involved screen for swallow every 3 months min dysphagia will progress PEG tube may be recommended; must be placed before pt vital capacity falls below 50% (pts can still aspirate with PEG tube)
29
when is non invasive ventilation recommended
when vital capacity decreases to 50% when this is no longe tolerated/effective pt will either get a trach or move to hospice management
30
medical management for communication with ALS
SLP involved early intervention: -over exaggerate words - speak slow - environment changes - quieter as dysarthria worsens: - writing board - alphabet board - electronic assistive communication devices
31
medical management for cognition
screen at least once annually referral to neuropsychologist may be needed increase in caregiver burden if there is cognitive deficits outcome measures: - ALS cognitive behavioral scale (ALS-CBS) - Edinburgh Cognitive and Behavioral ALS Screen
32
disease specific outcome measures for ALS
ALS functioning rating scale (ALDFRS) ALS functioning rating scale revised (ALSFRS-R) - measures 12 aspects of physical function -ranges from ability to swallow and use utensils to climbing stairs and breathing -MCID = 20% decline is considered significant
33
early stages of ALS S&S
mild to mod mm weakness in specific mm groups increase in difficulty with mobility and ADLs at end stage
34
middle stages of ALS S&S
severe mm weakness in some mm groups; mild to mod in others increase WC use increase assist needed with ADLs pain
35
late stages of ALS S&S
severe weakness in all mm groups dependent on WC/bed bound dependent for ADLs dysarthria/dysphagia respiratory compromise pain
36
goals of PT for ALS
keep pt functioning safely at highest level possible for as long as possible improve QOL minimize fatigue minimize risk of injury to pt and family address current and future impairment ** keep in mind what is important to pt and fam
37
common impairments associated with ALS
cervical mm weakness pain UE mm weakness LE mm weakness/gait probs resp mm weakness difficulty with ADLs decreased mobility mm cramps/spasticity
38
presentation/treatment for cervical weakness associated with ALS
extensors will weaken/worsen with progression treatment = bracing/WC set up
39
pain presentation with ALS and treatment options
commonly due to secondary effects of disease process related to ROM, mobility, positioning, etc shoulder/back/neck pain are common treatments: - modalities - ROM/stretch - jt mobs - jt protection - proper WC/bed positioning
40
ways to help with UE weakness with ALS
adaptive equipment splints mobile arm support
41
ways to help LE weakness with ALS and things to consider
AFO or assistive devices common consider weight of device, safety, and rate of disease progression
42
what to edu pt/caregiver on regarding respiratory health
energy conservation techniques positioning to prevent aspiration breathing exercises manual assisted cough techniques
43
adaptive equipment common with ALS
no single piece is appropriate for all pts/stages AD use will increase as disease progresses involve caregivers when choosing finances may be limiting factor OTs are great resource when deciding
44
AD options when pt has difficulty with transfers
elevate seat height lift chairs slide board mechanical lift
45
mm cramp and spasticity management techniques for those with ALS
exercise splinting WC positioning
46
things to keep in mind in regard to exercise with ALS pts
weak or de-enervated mm are more susceptible to overwork damage exercise is only safe when it prevents disuse atrophy but does not cause over work damage research suggests that mod intensity exercise can be safe and beneficial (i.e. endurance and resistance)- study done on EARLY/MIDDLE stage ALS
47
S&S of overuse fatigue to watch out for when ALS pt is on exercise program
inability to perform ADLs increase mm cramps soreness increase fasciculations have pt keep exercise log use RPE scale to monitor exertion
48
exercise to AVOID with ALS pts
eccentric exercise high intensity exercise resistance training for mm with a grade of <3/5
49

Year 2: Neuromuscular II (18 decks)

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