ALS pt. 2 Flashcards

1
Q

classical ALS more common in who

A

men
2/3 of ALS population

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2
Q

classical ALS involves what

A

UMN and LMN
either limb onset or bulbar onset

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3
Q

early signs of classical ALS

A

progressive limb weakness, reduced dexterity, wasting of hands, muscle fasciculations

if bulbar - wasting of the tongue and muscles for speech and swallowing

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4
Q

limb onset first symptoms are due to what

A

result from damage to motor neurons in motor cortex - corticospinal

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5
Q

limb onset first symptoms look like what

A

distal to proximal
fasciculations, atrophy, weakness, spasticity

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6
Q

bulbar onset progression

A

faster than limb onset

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7
Q

bulbar onset first symptoms are due to what

A

damage to motor neurons in brainstem for corticobulbar tracts

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8
Q

bulbar onset first symptoms are due to what

A

CN IX to XII causing dysarthria and dysphagia
tongue atrophy, tongue fasciculations
vocal cord spasms

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9
Q

bulbar onset is more common in who

A

older females

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10
Q

primary lateral sclerosis

A

UMN SxS only
can progress to classical

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11
Q

key examination findings for primary lateral sclerosis include

A

spasticity
UMN pattern weakness
pseudobulbar findings

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12
Q

prognosis for primary lateral sclerosis

A

relatively good with life expectancy

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13
Q

progressive spinal muscular atrophy mainly affects who

A

men
21 y/o

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14
Q

progressive spinal muscular atrophy (SMA IV)

A

LMN only
mainly limb
may limit to one region

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15
Q

SMA IV progression

A

variable - slow or rapid

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16
Q

SMA IV survival rate

A

if slow progressed - 5-10 yrs
survival duration 12 months longer

17
Q

flail arm syndrome

A

SMA IV

cervical
man in a barrel

18
Q

flail leg syndrome

A

SMA IV

lumbosacral

19
Q

how common is ALS with frontotemporal dementia

A

20-30% show cog symptoms

20
Q

what does the clinical course look like with ALS with FTD

A

more rapid

21
Q

familia ALS

A

combined effects of multiple predisposing + environmental & random effects leading to disease onset

22
Q

familia ALS onset

A

late forties or early fifties