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ALS Flashcards

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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
Radicava: form, MOA, SE, and outcomes
IV infusions unknown MOA, but antioxidant may cause allergic reaction slows decline in physical functioning
26
pts may take meds for symptom management such as:
pain spasticity sialorrhea anxiety depression
27
what are 2 medical management treatments under investigation
stem cell therapy gene therapy
28
Relyvrio: efficacy, MOA, ADE
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
29
what are 3 other medical management treatments/strategies needed for secondary support
respiratory care nutritional support brain computer interface
30
when may nutritional support be needed
earlier w bulbar onset bc ms involved in chewing/swallowing - later in limb onset
31
what does a brain computer interface offer and where is this seen
permits communication, operation of lights, computer - not in early dev in clinical trials
32
what are examples of respiratory care that individuals may need and why
CPAP or BiPAP assisted cough devices mechanical vent often have sleep apnea need cough assistive device for effective coughs to prevent pneumonia
33
when does someone finally present w ALS and why
once 80% of motor neurons lost - cover up sx for so long bc of neuroplasticity and the sprouting of intact dendrites
34
what are the most common presenting sx (3) and what does the pt often c/o
isolated weakness** ms cramping ms fasciculations "i kept tripping"
35
what clinical onset pattern is the most common
limb onset (70%) - bulbar is 30%
36
how and when do sensory deficits present
vague sensory changes in later stages in 20% sensation is usually intact bc sensory pathways are intact - not a hallmark
37
what is fronto-temporal dementia and how common is it
characterized by behavior changes, emotional dysregulation, involves motor functions - d/t loss of Betz cells in frontal lobe only in 30% of people
38
what are ANS involvement presentations and how common is it
irregularities in BP, HR, thermoregulation, sweating (40% of people)
39
what are the primary types of clinical s/sx seen
LMN and UMN
40
what are LMN s/sx seen (5)
weakness hyporeflexia hypotonicity atrophy ms cramps and fasciculations
41
what are UMN s/sx seen (4)
**spasticity** - more common than hypotonicity pathological reflex (babinski) hyperreflexia impaired motor control - inability to voluntary contract a ms
42
what are secondary s/sx
fatigue wt loss cachexia loss of ROM balance disturbance loss of mobility pressure ulcers contractures subluxation pain dec endurance depression anxiety
43
why is fatigue a common secondary s/sx
loss of motor neurons leads to energy inefficiency
44
why are pressure ulcers a possible secondary s/sx
repositioning difficulties issue w nutrition bc of problems w chewing/swallowing
45
what are bulbar s/sx (4)
dysarthria dysphagia sialorrhea pseudobulbar affect
46
what is pseudobulbar affect
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
what are respiratory s/sx (5)
dyspnea (DOE) nocturnal difficulty - CPAP accessory ms use dec cough effectiveness paradoxical breathing pattern
48
what are the 6 dz stages (per a typical limb onset)
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
how do the 6 dz stages look differently for a bulbar onset
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
what is the focus of PT management in ALS
focus on function and quality of life
51
given the progressive nature of the dz, how does this impact PT management
compensatory and preventative focus rather than restorative - planning for progression - better to be more aggressive
52
what is the purpose of exercise in ALS and what does research say
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
what are important things to avoid in exercise in ALS (4)
1. avoid high reps 2. avoid fatigue 3. shouldn't deplete energy available to perform daily, routine activities 4. avoid eccentric activities
54
what are suggested guidelines for exercises in ALS
mod intensity resistance in ms groups 3+/5 2/5 or less ms groups = PROM
55
why should cardiopulm exercises follow similar guidelines of mod intensity
even in early stages of ALS, there is an impaired VO2 max, work capacity and metabolic responses -> endurance will be limited
56
what is overwork damage and what should you do if you suspect this
physiological change if overwork weak ms -> can lead to new weaknesses that didn't have before exercise rest fully before return to exercise
57
what are 3 signs of overwork damage to monitor for
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
what are the 2 primary characteristics of early stages of ALS
mild weakness minimal difficulties w ADLs and mobility
59
what are the 2 strategies implemented for PT across all stages of ALS
restorative/prevention compensation
60
what are components in a restorative/prevention strategy in an early stage of ALS
strength - maintain max ms strength - prevent disuse atrophy - avoid overwork damage ROM: a/arom, stretching aerobic capacity and endurance
61
what are components in a compensatory strategy in an early stage of ALS
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
what are 4 primary characteristics of a middle stage of ALS
mobility and ADL decline mod weakness w/c use pain
63
what are components in a compensatory strategy in a middle stage of ALS
adaptive equipment, splints, orthotics, home modifications, w/c, caregiver training
64
what are components in a preventative strategy in a middle stage of ALS
a/arom to prom, stretching endurance pressure relief/skin protection
65
what are 7 primary characteristics of late stages of ALS
w/c dependent dependence w ADLs severe weakness and paralysis dysarthria dysphagia respiratory compromise pain
66
what are components in a compensatory strategy in a late stage of ALS
mechanical lift, ventilator support, caregiver ed and training, hospice or palliative care
67
what are components in a preventative strategy in a late stage of ALS
PROM pulmonary care/hygiene pressure relief and skin care
68
what is a battle that PT often has w insurance
insurance often denies coverage for equipment needed in later stages d/t short life expectancy
69
what are 3 disease specific standardized tests and measures? what type of measures are they all?
ALS functional rating scale ALS severity scale ALSAQ-40 all self report
70
what are 5 general standardized tests and measures that could be used and how do we determine when to utilize them
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
what are 7 components to ALS that an interdisciplinary approach addresses
spasticity management pain control augmented communication pulmonary hygiene nutrition psych support palliative care

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