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Neruo 1 - Test 3 > ALS > Flashcards

ALS Flashcards

1
Q

ALS

A

amyotrophic lateral sclerosis

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2
Q

ALS is also known as

A

lou gehrigs dz

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3
Q

what is ALS

A

most common form of adult onset progressive motor neuron dz

may be the most devastating of the neurologic degenerative dz

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4
Q

hallmark of ALS

A

combo of UMN and LMN signs and symptoms

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5
Q

UMN signs

A

spasticity

hyperreflexia

pathological reflexes

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6
Q

where do UMN signs stem from

A

destruction of motor neurons int he brain

causing degeneration of the corticospinal and corticobulbar tracts

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7
Q

LMN signs

A

muscle wasting

weakness

fasciculations

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8
Q

muscle weakness

A

amyotrophy

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9
Q

LMN signs stem from

A

destruction of the anterior horn cells of teh SC and cell bodies of the motor CN nuclei

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10
Q

90% of the CST fibers

A

decussate at the medulla

descend in the SC as the lateral CST

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11
Q

lateral sclerosis

A

destruction and scarring of the lateral CST

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12
Q

classification

A

sporadic

familial

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13
Q

sporadic

A

the most common form of ALS in the US

90-95% of all cases

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14
Q

familial

A

occurring more than once in family lineage (autosomal dominant)

accounts for very small number of cases in the US (5-10%)

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15
Q

familial is caused by

A

genetic mutation in a gene encoding an enzyme called copper-zinc superoxide dismutase

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16
Q

initial S&S

A

limb onset ALS

bulbar onset ALS

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17
Q

limb onset ALS

A

75% of cases

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18
Q

limb onset ALS is

A

difficulty walking or difficulty with the fine motor coordination of UEs

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19
Q

bulbar onset ALS

A

25% of cases

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20
Q

bulbar onset is

A

dysphagia

dysarthria

overactive gag reflex

21
Q

what motor nuclei are involved in bulbar

A

CN 5,7,9,10,12

22
Q

weakness and atrophy –> bulbar onset

A

tongue, pharynx, larynx, soft palate, muscles of the mouth and muscles of mastication

23
Q

what CN are typically spared –> bulbar onset

A

3,4,6

24
Q

pseudobulbar affects

A

15-45% of bulbar onset

25
Q

3 stages of diagnosis

A

clinically possible

clinically probable

clinically definite

26
Q

clinically possible

A

UMN and LMN findings in the same region

27
Q

clinically probable

A

UMN and LMN findings in 2 regions

28
Q

clinically definite

A

UMN and LMN findings in 3 or more regions

29
Q

regions

A

bulbar

cervical

thoracic

lumbosacral

30
Q

dx is supported by

A

spread of signs and symptoms from one region to another

with a linear increase in severity

31
Q

dx can be ruled out by

A

presence of cognitive, oculomotor, B&B or sensory sx

32
Q

how to dx

A

exclusion

no definite tests for it

33
Q

exclusion tests

A

CSF

CT/MRI

biopsy or ms or nerve

lab tests

34
Q

other motor neuron dz

A

spinal muscle atrophy

primary lateral sclerosis

35
Q

spinal muscle atrophy

A

autosomal recessive genetic LMN dz of childhood

36
Q

primary lateral sclerosis

A

slowly progressive UMN dz

characterized by spasticity and hyperreflexia

37
Q

onset of primary lateral sclerosis

A

after 40

38
Q

is PLS an subset of ALS

A

unknown

39
Q

incidence

A

4-6/100000

40
Q

incidence gender

A

men > women by 2:1

except bulbar onset affects females and older people more

41
Q

90% of ALS occurs

A

sporadically with an unknown cause

42
Q

mean age at onset

A

57

43
Q

clinical presentation

A

no lab test to confirm dx

nerve conduction velocity is normal

emg consistent with LMN dz

44
Q

dz is based on

A

recognition of clinical pattern or syndrome

one of exclusion

45
Q

first and earliest symptoms notes in 90% of pt

A

striated muscle weakness

initially focal but grows to include the entire body

46
Q

onset is

A

insidious

with most pts being unaware of changes until they have difficulty with fxnal activity

47
Q

physical exam

A

MMT will show greater weakness than the pt reports

48
Q

what occurs by the time pt c/o weakness

A

up to 80% of the motor neurons int heir areas of weakness have been lost

Neruo 1 - Test 3 (12 decks)

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