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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
Q

negative prognostic factors for ALS

A

bulbar onset

executive dementia

psychological distress

26
Q

positive predictive factors for ALS

A

younger age of onset

limb onset ALS

delay between symptom onset and diagnosis

27
Q

pharmacology management options for ALS and the side effects of each

A

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
Q

med management for dysphagia

A

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
Q

when is non invasive ventilation recommended

A

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
Q

medical management for communication with ALS

A

SLP involved

early intervention:
-over exaggerate words
- speak slow
- environment changes - quieter

as dysarthria worsens:
- writing board
- alphabet board
- electronic assistive communication devices

31
Q

medical management for cognition

A

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
Q

disease specific outcome measures for ALS

A

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
Q

early stages of ALS S&S

A

mild to mod mm weakness in specific mm groups

increase in difficulty with mobility and ADLs at end stage

34
Q

middle stages of ALS S&S

A

severe mm weakness in some mm groups; mild to mod in others

increase WC use

increase assist needed with ADLs

pain

35
Q

late stages of ALS S&S

A

severe weakness in all mm groups

dependent on WC/bed bound

dependent for ADLs

dysarthria/dysphagia

respiratory compromise

pain

36
Q

goals of PT for ALS

A

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
Q

common impairments associated with ALS

A

cervical mm weakness
pain
UE mm weakness
LE mm weakness/gait probs
resp mm weakness
difficulty with ADLs
decreased mobility
mm cramps/spasticity

38
Q

presentation/treatment for cervical weakness associated with ALS

A

extensors will weaken/worsen with progression

treatment = bracing/WC set up

39
Q

pain presentation with ALS and treatment options

A

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
Q

ways to help with UE weakness with ALS

A

adaptive equipment

splints

mobile arm support

41
Q

ways to help LE weakness with ALS and things to consider

A

AFO or assistive devices common

consider weight of device, safety, and rate of disease progression

42
Q

what to edu pt/caregiver on regarding respiratory health

A

energy conservation techniques

positioning to prevent aspiration

breathing exercises

manual assisted cough techniques

43
Q

adaptive equipment common with ALS

A

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
Q

AD options when pt has difficulty with transfers

A

elevate seat height

lift chairs

slide board

mechanical lift

45
Q

mm cramp and spasticity management techniques for those with ALS

A

exercise

splinting

WC positioning

46
Q

things to keep in mind in regard to exercise with ALS pts

A

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
Q

S&S of overuse fatigue to watch out for when ALS pt is on exercise program

A

inability to perform ADLs
increase mm cramps
soreness
increase fasciculations

have pt keep exercise log

use RPE scale to monitor exertion

48
Q

exercise to AVOID with ALS pts

A

eccentric exercise

high intensity exercise

resistance training for mm with a grade of <3/5

49
Q
A

Year 2: Neuromuscular II (18 decks)

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