ALS Flashcards

1
Q

when is the average age of onset

A

mid 50s

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2
Q

what is the pathogenesis of ALS and what makes it unique among neuro conditions

A

progressive degenerative motor neuron dz

has features of both LMN and UMN conditions

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3
Q

what is the pathophys that leads to features of both UMNs and LMNs

A

LMN: loss of ant horn cells and CN motor nuclei w degeneration of axons -> denervated skeletal ms and atrophy (AMMYOTROPHIC)

UMN: degeneration of Betz cells in motor cortex w demyelination and gliosis of corticospinal (LATERAL SCLEROSIS) and corticobulbar tracts
- tracts fill w plaque and scar
- tracts run laterally = lateral sclerosis
- corticobulbar = impact CNs

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4
Q

what CNs are typically spared

A

III, IV, and VI
- eye mvmts preserved

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5
Q

what tract and function is often spared

A

spinocerebellar tracts and sensory function

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6
Q

what other physiological changes also happen in the CNS and is this considered primary or secondary to ALS pathogenesis

A

mitochondrial dysfunction, collection of cytoskeleton proteins, high levels of glutamate (excitatory neurotransmitter in CNS), free radical release (garbage in CNS), excitotoxicity, inflammation and apoptosis

not sure if secondary to dz process or causing it

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7
Q

what are the two patterns of onset

A

bulbar onset
limb onset

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8
Q

describe a bulbar pattern of onset

A

difficulty speaking, swallowing, respiratory w onset (CNs)

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9
Q

describe a limb pattern of onset

A

asymmetrical weakness in UE, LE, or both
will eventually have impairments in both as dz progresses

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10
Q

who do you see a slower progression in

A

younger pts
limb onset

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11
Q

what is the prognosis but what about this is slightly misleading

A

poor, death w/i 2-5 years after dx

dz process has been likely occurring for several years before dx bc of the damage seen when finally dx

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12
Q

what is the most common cause of death in ALS

A

respiratory complications
- respiratory ms so weak can’t breathe -> respiratory failure

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13
Q

what are some etiologies

A

not well understood
cell abnormalities triggered by genetic predisposition and/or environmental insults

5-10% have genetic link
mutation of chromosome 21
theories: viral infection, environmental toxins, autoimmune reaction

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14
Q

how could a gene mutation of chromosome 21 be a possible etiology and what does the research say

A

dec action of superoxide dismutase 1
- enzyme/protein that helps to breakdown garbage and byproducts of O2 metabolism

leads to free radical accumulation -> start cytotoxicity process

carriers of mutation don’t always develop ALS

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15
Q

how is ALS dx

A

no definitive dx test

dx made by recognition of clinical pattern (both UMN and LMN signs) and a synthesis of test results
- neuroimaging (MRI), SPECT/PET

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16
Q

how may neuroimaging (ie MRI) help w dx

A

may show signs of atrophy of precentral and postcentral gyri, frontal lobe
may see scarring in descending corticospinal tracts

may be normal especially in early stages

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17
Q

how may SPECT/PET help w dx

A

evidence of glucose hypometabolism c/w neuronal loss in motor cortex & corticospinal tract
- more sensitive in detection

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18
Q

what may PET and fMRI show in dx and why would this be the case

A

shift of motor cortical activation and extra-motor activation during limb task
- neuroplasticity able to reshape cortex and take over new functions as Betz cells degenerate

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19
Q

how might EMGs help in dx

A

spontaneous fibrillations and fasciculations w giant or large unit spikes upon volitional activity

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20
Q

how might NCV help in dx

A

usually WNL
- if nerve hasn’t degenerated yet, conduction isn’t impaired

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21
Q

how might a CSF sample help in dx

A

inflammatory markers, elevated proteins may be present
- no specific biomarkers determined yet

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22
Q

what has been a major limitation in trying to develop effective meds

A

short lifespan after dx

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23
Q

what are limitations in medical management (4)

A

poorly understood etiology
different phenotypes
difficult to dx early
lack of translation b/w mouse and human models

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24
Q

what are properties of Riluzole and what were outcomes

A

glutamate inhibitor
- dec amt of glutamate in CNS
neuroprotective agent
- dec hyperexcitability of neurons and damage from accumulation of glutamate

inc survival by ~3mo

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25
Q

Radicava: form, MOA, SE, and outcomes

A

IV infusions
unknown MOA, but antioxidant
may cause allergic reaction
slows decline in physical functioning

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26
Q

pts may take meds for symptom management such as:

A

pain
spasticity
sialorrhea
anxiety
depression

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27
Q

what are 2 medical management treatments under investigation

A

stem cell therapy
gene therapy

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28
Q

Relyvrio: efficacy, MOA, ADE

A

better survival than in other meds

supports survival of motor neurons by supporting mitochondria and sarchoplasmic reticulum

main ADE are GI sx
- diarrhea, abdominal pain, nausea
- upper respiratory tract infections

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29
Q

what are 3 other medical management treatments/strategies needed for secondary support

A

respiratory care
nutritional support
brain computer interface

30
Q

when may nutritional support be needed

A

earlier w bulbar onset bc ms involved in chewing/swallowing
- later in limb onset

31
Q

what does a brain computer interface offer and where is this seen

A

permits communication, operation of lights, computer
- not in early dev

in clinical trials

32
Q

what are examples of respiratory care that individuals may need and why

A

CPAP or BiPAP
assisted cough devices
mechanical vent

often have sleep apnea
need cough assistive device for effective coughs to prevent pneumonia

33
Q

when does someone finally present w ALS and why

A

once 80% of motor neurons lost
- cover up sx for so long bc of neuroplasticity and the sprouting of intact dendrites

34
Q

what are the most common presenting sx (3) and what does the pt often c/o

A

isolated weakness**
ms cramping
ms fasciculations

“i kept tripping”

35
Q

what clinical onset pattern is the most common

A

limb onset (70%)
- bulbar is 30%

36
Q

how and when do sensory deficits present

A

vague sensory changes in later stages in 20%

sensation is usually intact bc sensory pathways are intact
- not a hallmark

37
Q

what is fronto-temporal dementia and how common is it

A

characterized by behavior changes, emotional dysregulation, involves motor functions
- d/t loss of Betz cells in frontal lobe

only in 30% of people

38
Q

what are ANS involvement presentations and how common is it

A

irregularities in BP, HR, thermoregulation, sweating

(40% of people)

39
Q

what are the primary types of clinical s/sx seen

A

LMN and UMN

40
Q

what are LMN s/sx seen (5)

A

weakness
hyporeflexia
hypotonicity
atrophy
ms cramps and fasciculations

41
Q

what are UMN s/sx seen (4)

A

spasticity
- more common than hypotonicity
pathological reflex (babinski)
hyperreflexia
impaired motor control
- inability to voluntary contract a ms

42
Q

what are secondary s/sx

A

fatigue
wt loss
cachexia
loss of ROM
balance disturbance
loss of mobility
pressure ulcers
contractures
subluxation
pain
dec endurance
depression
anxiety

43
Q

why is fatigue a common secondary s/sx

A

loss of motor neurons leads to energy inefficiency

44
Q

why are pressure ulcers a possible secondary s/sx

A

repositioning difficulties
issue w nutrition bc of problems w chewing/swallowing

45
Q

what are bulbar s/sx (4)

A

dysarthria
dysphagia
sialorrhea
pseudobulbar affect

46
Q

what is pseudobulbar affect

A

when degeneration in frontal lobe, can see spontaneous onset of laughing/crying w no emotional triggers
- will often feel embarrassed by this -> lead to activity limiting behaviors w isolation

47
Q

what are respiratory s/sx (5)

A

dyspnea (DOE)
nocturnal difficulty - CPAP
accessory ms use
dec cough effectiveness
paradoxical breathing pattern

48
Q

what are the 6 dz stages (per a typical limb onset)

A
  1. early, (I), weak in specific ms groups
  2. early, mod weakness in ms groups, minor difficulty w mobility/fine motor/ADLs
  3. middle, mild to mod limitation in mobility, ambulatory w orthotics/ADs
  4. middle, severe limb weakness, w/c for mobility, MI w ADLs using ADs
  5. late, w/c for all mobility, dependent transfers & ADLs
    - pain and pressure ulcers
  6. late, dependent for all mobility and care, respiratory complications, contractures, pain
49
Q

how do the 6 dz stages look differently for a bulbar onset

A
  1. hoarseness, aspiration
  2. more difficulty speaking, swallowing
  3. change in food textures, thickened liquids

similar sx stages 4-6 bc will have limb sx at this point

50
Q

what is the focus of PT management in ALS

A

focus on function and quality of life

51
Q

given the progressive nature of the dz, how does this impact PT management

A

compensatory and preventative focus rather than restorative
- planning for progression
- better to be more aggressive

52
Q

what is the purpose of exercise in ALS and what does research say

A

won’t change rate of progress, but will help w quality of life

there isn’t a lot of evidence, and not a lot of guidelines as a result

53
Q

what are important things to avoid in exercise in ALS (4)

A
  1. avoid high reps
  2. avoid fatigue
  3. shouldn’t deplete energy available to perform daily, routine activities
  4. avoid eccentric activities
54
Q

what are suggested guidelines for exercises in ALS

A

mod intensity resistance in ms groups 3+/5

2/5 or less ms groups = PROM

55
Q

why should cardiopulm exercises follow similar guidelines of mod intensity

A

even in early stages of ALS, there is an impaired VO2 max, work capacity and metabolic responses
-> endurance will be limited

56
Q

what is overwork damage and what should you do if you suspect this

A

physiological change if overwork weak ms
-> can lead to new weaknesses that didn’t have before exercise

rest fully before return to exercise

57
Q

what are 3 signs of overwork damage to monitor for

A
  1. inc fatigue the same or following day that is different from typical end of day fatigue
  2. functional weakness or weakness that prevents them from performing an activity they could do prior
  3. inc ms soreness, cramping, or fasiculations
58
Q

what are the 2 primary characteristics of early stages of ALS

A

mild weakness
minimal difficulties w ADLs and mobility

59
Q

what are the 2 strategies implemented for PT across all stages of ALS

A

restorative/prevention
compensation

60
Q

what are components in a restorative/prevention strategy in an early stage of ALS

A

strength
- maintain max ms strength
- prevent disuse atrophy
- avoid overwork damage

ROM: a/arom, stretching

aerobic capacity and endurance

61
Q

what are components in a compensatory strategy in an early stage of ALS

A

adaptive equipment, ADs, home and work modifications, energy conservation
- teach them how to use equipment even if don’t need it yet (they will)

62
Q

what are 4 primary characteristics of a middle stage of ALS

A

mobility and ADL decline
mod weakness
w/c use
pain

63
Q

what are components in a compensatory strategy in a middle stage of ALS

A

adaptive equipment, splints, orthotics, home modifications, w/c, caregiver training

64
Q

what are components in a preventative strategy in a middle stage of ALS

A

a/arom to prom, stretching
endurance
pressure relief/skin protection

65
Q

what are 7 primary characteristics of late stages of ALS

A

w/c dependent
dependence w ADLs
severe weakness and paralysis
dysarthria
dysphagia
respiratory compromise
pain

66
Q

what are components in a compensatory strategy in a late stage of ALS

A

mechanical lift, ventilator support, caregiver ed and training, hospice or palliative care

67
Q

what are components in a preventative strategy in a late stage of ALS

A

PROM
pulmonary care/hygiene
pressure relief and skin care

68
Q

what is a battle that PT often has w insurance

A

insurance often denies coverage for equipment needed in later stages d/t short life expectancy

69
Q

what are 3 disease specific standardized tests and measures? what type of measures are they all?

A

ALS functional rating scale
ALS severity scale
ALSAQ-40

all self report

70
Q

what are 5 general standardized tests and measures that could be used and how do we determine when to utilize them

A

sickness impact profile (SIP)
FIM-inpatient rehab only
fatigue severity scale
2 or 6min walk test
- earlier stages and amb
10m walk test

dependent on client and goals

71
Q

what are 7 components to ALS that an interdisciplinary approach addresses

A

spasticity management
pain control
augmented communication
pulmonary hygiene
nutrition
psych support
palliative care