ALS Flashcards

1
Q

What is the most common adult onset MND?

A

motor neuron disease is ALS

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2
Q

What does ALS stand for?

A

Amyotrophic Lateral sclerosis

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3
Q

How is ALS characterized?

A

progressive
fatal
paralytic muscle wasting

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4
Q

What nuero system is involved in ALS?

A

UMN & LMN

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5
Q

What is age onset of ALS? and average onset

A

teens to 80’s average 50’s

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6
Q

What are the two types of ALS? and %?

A

SALS (sporadic) 90-95%

FALS (familial) 10-5%

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7
Q

What are the two onsets of ALS? and %?

A

limb onset 70-80%

bulbar onset 20-30%

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8
Q

What are limb and bulbar onset ALS?

A

limb onset: initial onset in the extremities and UMN>LMN

bulbar onset: initial involvement of bulbar muscles ie muscles of face and throat and LMN>UMN

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9
Q

Which onset if more detrimental?

A

bulbar affecting breathing

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10
Q

ALS male or female more common?

A

male ALS

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11
Q

What are some possible reasons for ALS?

A

oxidative stress
neurotoxicity (mercury, lead)
protein aggregation
lifestyle factors

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12
Q

What are common risk factors for ALS?

A
mutation: SOD1
Cluster of demographic: western Pacific male
Gender: male>female
Age
Family hx
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13
Q

Disease course of ALS?

A

most die within 3-5 years due to respiratory failure

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14
Q

What is Pseudobulbar Palsy? S&S?

A

Corticobulbar tract affected
Speech, mastication, swallow
Pseudobulbar effect

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15
Q

What is Pseudobulbar effect?

A

sudden crying and laughing

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16
Q

What is Progressive Bulbar Palsy? S&S?

A

CN affected
Swallow, mastication, facial muscles
Fasciculation of tongue PROMINENT

17
Q

What is PLS? S&S?

A

Primary lateral Sclerosis
Corticospinal tract, neuron loss in cortex
ONLY UMN affected: hyperreflexia/ spasticity
no fasciculation or atrophy

18
Q

What is PMA? S&S?

A

Progressive Muscular Atrophy
loss of motor neurons in Anterior horn cells
ONLY LMN
progressive weakness, wasting, & fasciculation of hand muscles

19
Q

MIX of what two forms results in the term ALS?

A

PLS & PMA= ALS

20
Q

What are the best indicator to survival in ALS?

A

age at time of onset,
<35 y.o has best survival rate than older pt
and
limb-onset: they have better outcome than bulbar

21
Q

What are some UMN signs?

A
spastic
hyperreflexia
clonus
Babinski
muscle weakness
22
Q

What are some LMN signs?

A
weakness
focal
asymmetrical
begins in UE, LE, or Bulbar muscles
Begins distally in extremities
hyporeflexia, hypotonia, fatigue
23
Q

What is a typical weakness in LMN ALS?

A

C/S extensors

24
Q

What are some Bulbar signs?

A
Dysarthria
Dyphagia
Sialorrhea
Pseudobulbar effect
respiratory dysfunction
25
Q

What is Dysarthria?

A

hoarseness or slurring

26
Q

What is Sialorrhea?

A

drooling

27
Q

What are common cognitive S&S in ALS?

A

frontotemporal dementia

behavior changes

28
Q

What medication is for ALS?

A

Rilotek: extends survival by 2-3 months

29
Q

What are some rehab considerations for ALS?

A

strengthening involved muscles won’t improve
do not overtrain/fatigue
rehab: flexibility and aerobic