ALS Flashcards

1
Q

what is the most common and fatal motor neuron disease

A

ALS

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2
Q

mortality rate

A

2-5 years
variable

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3
Q

what is ALS characterized by

A

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

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4
Q

what is specifically degenerated

A

anterior horn cells
corticobulbar tracts
corticospinal tracts

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5
Q

what is onset

A

> 50 years

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6
Q

men or women are more affected

A

men, slightly

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7
Q

what causes the UMN symptoms

A

degeneration of UMN cell bodies in motor cortex

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8
Q

what causes the LMN symptoms

A

alpha motor neurons LMN of spinal cord

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9
Q

what is one cause of ALS

A

glutamate excitotoxicity

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10
Q

what does glutamate excitotoxicity increase

A

increased CSF, plasma, post-mortem tissue

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11
Q

risk factors for ALS

A

male > female
caucasian, non-hispanic
geographical clusters
family hx
55-75 y/o
priot trauma / TBI

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12
Q

diagnosis of ALS

A

difficult
dx by exclusion*
clinical exam
EMG/NCV
MRI
CSF, blood and urine tests – high creatine phosphokinase levels

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13
Q

what does a clinical dx of ALS require

A

pattern of observed and reported symptoms of both UMN/LMN

persistent decline in physical fxn that can’t be attributed to other disorders

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14
Q

what is the El escorial criteria for dx of ALS

A

exclusion of all other dx + progressive functional decline + progressive UMN + LMN deficits in more than or at least 1 region of the body

OR

LMN deficits in more than or at least 1 limb/region as defined by clinical examination and/or EMG in 2 clinical regions

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15
Q

what regions are involved with El escorial criteria

A

defined as bulbar, cervical, thoracic, lumbosacral

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16
Q

what is included in the gold coast criteria for dx of ALS

A

investigations excluding other disease processes AND
progressive motor impairment documented by hx or repeated clinical assessment, preceded by normal fxn AND UMN/LMN signs in at least 1 region of the body OR LMN dysfunction in at least 2 regions

17
Q

what regions are involved with gold coast criteria for dx of ALS

A

bulbar, UE/LE

18
Q

what does El escorial criteria dx lead to

A

suspected, possible, probable and definite ALS

19
Q

what does gold coast criteria for dx lead to

A

+ ALS or - ALS

20
Q

cardinal sign of ALS

A

muscle weakness - focal, asymmetrical muscle weakness beginning in LE or UE or bulbar weakness

21
Q

do you see more UMN weakness or LMN

A

LMN weakness

22
Q

what happens to eye, bowel and bladder

A

normal spared until terminal stages

23
Q

what do respiratory impairments look like for this population

A

decreased strength
reduction of VC (25-35%)
early S&S of muscle weakness

24
Q

what do cognitive impairments look like for this population

A

variable
verbal fluency, language comprehension, memory, abstract reasoning

25
who are cognitive impairments more common in
bulbar onset
26
additional impairments
pain fatigue ANS symptoms
27
what is spared in this disease
sensory pathways ocular motor fxn sphincter (bowel/bladder)