ALS Flashcards

1
Q

what is ALS

A

a neurodegenerative disease that effects the motor neuros of the BS, brain and the spinal cord

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2
Q

what is the most common neuro disease in the adult population

A

ALS

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3
Q

what age does ALS occur at

A

at any age but normally after 50s

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4
Q

when does death occur for these indivduals

A

3-5 from onset

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5
Q

what type of ALS is more prominent limb or bulbar

A

limb onset
70% - 80%

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6
Q

how prevent is the bulbar onset of ALS

A

20 - 30%

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7
Q

Limb-onset ALS presentation

A

Initial involvement in extremities

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8
Q

Bulbar-onset presentation

A

initial involving the bulbar muscle - diffucty speaking, chewing, and swallowing

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9
Q

what pop is the bulbar more prevalent in

A

middle aged women

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10
Q

how many people with ALS have a family presentation with it

A

5 -10%

Autosomal dominant (in most cases)

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11
Q

hat are known risk factors for ALS

A

disease causing mutations

clusters - western pacific ALS

family history

age

gender (male > females)

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12
Q

possible risk factors

A

neurotoxiant disorders

certian occupations

diet

lifestyle factors

vigours physical axtivity

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13
Q

what is the pathophysiology of ALS

A

progressive degeneration and loos of motor neurons in the spinal cord, motor cortex, and motor cortex

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14
Q

early versus late in the disease progression neurosn

A

early - healthy intaxct neurons can replace the degenerated neurons

later - there are not more healthy neuron to replace the degenerated ones

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15
Q

what areas impact with ALS

A

UMN

corticospinal tract

brainstem nuclei for CN 5, 7, 9, 10, 12

anterior horn of the spinal cord

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16
Q

what is normally spared with ALS

A

brainstem nuclei that are controlling the extral ocular movemnts

motor nuclei that are controlling the pelvic floor striated muscles - anal and external sphincter

sensory system

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17
Q

what is the most frequently presenting impairment

A

focal assymetrical weakness

muscle weakness is isolated to. a specific muscle

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18
Q

what is the caridnal sign of ALS

A

muscle weakness

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19
Q

LMN path

A

muscle weakness

hyporeflexia

hypotonicity

atrophy

muscle cramp

faciulations

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20
Q

what is a fasciculation

A

a muscle twitch

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21
Q

UMN patho

A

hyperreflexia

hypertonicty - spacity

muscle weakness

patho relfexes

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22
Q

bulbar patho

A

dysphalgia

dysarrthia

sialorrhea

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23
Q

what is sialorrhea

A

excessive saliva flow

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24
Q

what is what is pseudobulbar affect

A

Inappropriate involuntary laughing and crying due to a nervous system disorder.

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25
Respiratory impairments seen with ALs
Inspiratory/expiratory muscle weakness, dyspnea/DOE, nocturnal respiratory difficulty, orthopnea, hypoventilation, secretion retention, ineffective cough
26
what is orthopneas
Discomfort when breathing while lying down flat;
27
neck issues and ALS
neck stiffness heavy head sensation hard time stablizing the head
28
what are 2nd impairment that you might see with muscle weakness
contracture sublexation tendon shortening decreased ROM adhesive capcilitus amb difficulties foot drop
29
what symptoms do we look for the diagnosis of ALS
LMN degradation - neuroexamination UMN degration progressive spread if signs within the specfic region
30
what must be absent for the issue to be ALS
Electrophysiologic evidence of other disease processes that might explain the signs of LMN or UMN degeneration Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiologic signs
31
how does ALS spread
initialing the sym spread withing the segment of onset then to other regions
32
spread for unilateral arm onset
contra arm isp leg contra leg bulbar muscles
33
spread for uni leg
contra leg isp arm contra arm bulbar muscles
34
spread for bulbar onset
one arm contra arm
35
what is death normally due to
respiratory failure
36
do younger people have better survial rate then older people
yes
37
Delay between symptom onset and diagnosis leads to what
increase mortality
38
what does the presence of executive dementia lead to
worse prognosis
39
Less severe involvement at time of diagnosis leads to what kind of prognosis
better prognosis
40
what goes into the management of ALS
pt and family edu management of symptoms nutrition management respiratory decisions cog drug therapies pallative care
41
early stage ALS what are we working on as a PT
preventative restorative compensatory
42
middle/late stage ALS what are we working on as a PT
compensatory preventative resortative
43
are there drig therapies for ALS
yes Riluzole, Edarvone, Relyvrio
44
what do you prioritize on the PT neuro exam
CN Motor function balance
45
what do you screen on the PT neuro exam
cognition sensory coordination - should be intact since the cere is spared
46
what is the The Revised ALSFRS (ALSFRS-R)
0 - worst 48 - best correlate significantly with quality of life as measure
47
what is the ALS Assessment Questionnaire
Measure subjective health status (impairments and disabilities) Physical mobility, ADL’s, eating and drinking, communication and emotional functioning 0-100 scale (worst - best)
48
Restorative PT interventions
improve impairments and activity modifications
49
when is it good to use restrorative methods
middle/early stages these are only temp solutions
50
Compensatory PT interventions
lost something so use this instead modify activity, task, or enviroment to minimize activity limitation and participation restrictions
51
what is preventative PT interventions
min potential impairments - loss of ROM or strengt, pneumonia,
52
what restorative/preventative activities do we do in the early stages of ALS
strengthing endurance stretching - ROM , AAROM
53
wheel chair use in the middle stage of ALS
WC is needed for longer distances and all of the time towards the end of this stage
54
muscle weakness seen in the middle stage
serve muscle weakness in some group and minor in other groups
55
late stage WC use
WC dependent
56
late stage ADLs
completely dependent
57
lates stage muscle strength
weakness everywhere -repirtory compromise
58
what is the main roll of the PT throughout the stage of ALS
promoting functional independence promoting health and wellness providing alternative means for carrying out difference functional tasks min complications edu and support
59
through what stages is the person with ALS able to amb
stage 1 -3 AD come in at stage 2
60
stage 3 weakness
server weakness in certain groups
61
at what stage is the ALS pt dep
stage 5
62
at what stage is the ALS bedridden
stage 6
63
intervention progress - early stage
edu aerobic and strengthening activity energy conservation stage
64
intervention progress - middle
DME if needed transition to PROM/AROM plan from a resitive plan education energy conservation family training
65
intervention progress - late
edu DME PROM for comfort prevention of PNA and pressure injuries
66
what to do for cervical weakness
neck braces supportive seating (high backs) elevated reading materials tilit in space WCs education about good arm support for long sitting periods
67
for dsyarthria and dysmetria what to do
SLP heand and trunk control chin tuck for swallowing edu about aspiration risk
68
what to do about respiratory muscle weakness
energy conservation tech sign of apiration sign for resp infection oral secreation and choking episodes
69
why are Orthoses good
support weak muscle reduce stress on functional muscles energy conservation mim fatigue
70
what are some interventions for muscle cramps and spascity
soft tissue mobilization stretching cold (short term reduction in spac) edu about slow prolonged stretches postural positioning splints
71
what is normally included in a PT treatment plan for ALS
ROM and stretching low to moderated resistance training to functional muscles submax aerobic activities
72
what exercises should we avoid with ALS
highly rep or high resistant exercises
73
can you strengthen muscle that have been weakened by ALS
no no pain no gain does not work here
74
how often should the exercise routine be done
daily can be broken into part to avoid aftigue
75
what level of exercise should be perfromed
mod intensity low resistance