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
Q

Respiratory impairments
seen with ALs

A

Inspiratory/expiratory muscle weakness,

dyspnea/DOE,

nocturnal respiratory difficulty, orthopnea,

hypoventilation,

secretion retention, ineffective cough

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26
Q

what is orthopneas

A

Discomfort when breathing while lying down flat;

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27
Q

neck issues and ALS

A

neck stiffness

heavy head sensation

hard time stablizing the head

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28
Q

what are 2nd impairment that you might see with muscle weakness

A

contracture

sublexation

tendon shortening

decreased ROM

adhesive capcilitus

amb difficulties

foot drop

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29
Q

what symptoms do we look for the diagnosis of ALS

A

LMN degradation - neuroexamination

UMN degration

progressive spread if signs within the specfic region

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30
Q

what must be absent for the issue to be ALS

A

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

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31
Q

how does ALS spread

A

initialing the sym spread withing the segment of onset then to other regions

32
Q

spread for unilateral arm onset

A

contra arm

isp leg

contra leg

bulbar muscles

33
Q

spread for uni leg

A

contra leg

isp arm

contra arm

bulbar muscles

34
Q

spread for bulbar onset

A

one arm

contra arm

35
Q

what is death normally due to

A

respiratory failure

36
Q

do younger people have better survial rate then older people

A

yes

37
Q

Delay between symptom onset and diagnosis leads to what

A

increase mortality

38
Q

what does the presence of executive dementia lead to

A

worse prognosis

39
Q

Less severe involvement at time of diagnosis leads to what kind of prognosis

A

better prognosis

40
Q

what goes into the management of ALS

A

pt and family edu

management of symptoms

nutrition management

respiratory decisions

cog

drug therapies

pallative care

41
Q

early stage ALS what are we working on as a PT

A

preventative

restorative

compensatory

42
Q

middle/late stage ALS what are we working on as a PT

A

compensatory

preventative

resortative

43
Q

are there drig therapies for ALS

A

yes

Riluzole, Edarvone, Relyvrio

44
Q

what do you prioritize on the PT neuro exam

A

CN

Motor
function
balance

45
Q

what do you screen on the PT neuro exam

A

cognition

sensory

coordination - should be intact since the cere is spared

46
Q

what is the The Revised ALSFRS (ALSFRS-R)

A

0 - worst
48 - best

correlate significantly with quality of life as measure

47
Q

what is the ALS Assessment Questionnaire

A

Measure subjective health status (impairments and disabilities)

Physical mobility, ADL’s, eating and drinking, communication and emotional functioning

0-100 scale (worst - best)

48
Q

Restorative PT interventions

A

improve impairments and activity modifications

49
Q

when is it good to use restrorative methods

A

middle/early stages

these are only temp solutions

50
Q

Compensatory PT interventions

A

lost something so use this instead

modify activity, task, or enviroment to minimize activity limitation and participation restrictions

51
Q

what is preventative PT interventions

A

min potential impairments - loss of ROM or strengt, pneumonia,

52
Q

what restorative/preventative activities do we do in the early stages of ALS

A

strengthing

endurance

stretching - ROM , AAROM

53
Q

wheel chair use in the middle stage of ALS

A

WC is needed for longer distances and all of the time towards the end of this stage

54
Q

muscle weakness seen in the middle stage

A

serve muscle weakness in some group and minor in other groups

55
Q

late stage WC use

A

WC dependent

56
Q

late stage ADLs

A

completely dependent

57
Q

lates stage muscle strength

A

weakness everywhere -repirtory compromise

58
Q

what is the main roll of the PT throughout the stage of ALS

A

promoting functional independence

promoting health and wellness

providing alternative means for carrying out difference functional tasks

min complications

edu and support

59
Q

through what stages is the person with ALS able to amb

A

stage 1 -3

AD come in at stage 2

60
Q

stage 3 weakness

A

server weakness in certain groups

61
Q

at what stage is the ALS pt dep

A

stage 5

62
Q

at what stage is the ALS bedridden

A

stage 6

63
Q

intervention progress - early stage

A

edu

aerobic and strengthening activity

energy conservation stage

64
Q

intervention progress - middle

A

DME if needed

transition to PROM/AROM plan from a resitive plan

education

energy conservation

family training

65
Q

intervention progress - late

A

edu

DME

PROM for comfort

prevention of PNA and pressure injuries

66
Q

what to do for cervical weakness

A

neck braces

supportive seating (high backs)

elevated reading materials

tilit in space WCs

education about good arm support for long sitting periods

67
Q

for dsyarthria and dysmetria what to do

A

SLP

heand and trunk control

chin tuck for swallowing

edu about aspiration risk

68
Q

what to do about respiratory muscle weakness

A

energy conservation tech

sign of apiration

sign for resp infection

oral secreation and choking episodes

69
Q

why are Orthoses good

A

support weak muscle

reduce stress on functional muscles

energy conservation

mim fatigue

70
Q

what are some interventions for muscle cramps and spascity

A

soft tissue mobilization

stretching

cold (short term reduction in spac)

edu about slow prolonged stretches

postural positioning

splints

71
Q

what is normally included in a PT treatment plan for ALS

A

ROM and stretching

low to moderated resistance training to functional muscles

submax aerobic activities

72
Q

what exercises should we avoid with ALS

A

highly rep or high resistant exercises

73
Q

can you strengthen muscle that have been weakened by ALS

A

no

no pain no gain does not work here

74
Q

how often should the exercise routine be done

A

daily
can be broken into part to avoid aftigue

75
Q

what level of exercise should be perfromed

A

mod intensity

low resistance