Continuum PNS and MND: ALS and other

Question 1

Lifetime risk of having ALS

Answer 1

~1:400

Question 2

Sporadic ALS

Answer 2

60% genetic 40% environmental

Question 3

Occupations with risks

Answer 3

veterinarian, hairdressser, plant operator

Question 4

70% of ALS presents with

Answer 4

asymmetric distal weakness

Question 5

25% of ALS presents with

Answer 5

bulbar features

Question 6

UMN signs

Answer 6

spastic , slow, hyperreflexa, pathologic reflexes

Question 7

LMN signs

Answer 7

pure motor weakness, reduced reflexes, atrophy fasciculation and CRAMPS

Question 8

spastic dysarthria

Answer 8

slow and strained speech (UMN)

Question 9

flaccid dysarthria

Answer 9

weakness of lingual, facial and palatal muscles (breathy/hypernasal)

Question 10

Laryngospasm and cheek/tongue bitting

Answer 10

UMN signs

Question 11

sialorrhea

Answer 11

LMN signs

Question 12

Mixed spastic and flaccid dysarthria

Answer 12

almost always ALS

Question 13

Respiratory insufficiency primarily a

Answer 13

LMN degneration

Question 14

PBA localizes to

Answer 14

corticopontocerebellar pathways

Question 15

Cognitive difficult in ALS is usually

Answer 15

executive dysfunction

Question 16

Dx of als is based on

Answer 16

history exam EDX and exclusion of mimics

Question 17

El escorial and Awaji are not a measure of

Answer 17

disease severity

Question 18

Kings staging

Answer 18

ALS severity

Question 19

Sensory findings in ALS are considered

Answer 19

in proportion to the motor findings

Question 20

Early or slower progressive ALS has preserved…. because…

Answer 20

motor responses bc of collateral sprouting

Question 21

3 core elements of ALS DX

Answer 21

progressive, UMN and LMN

Question 22

4 dx categories

Answer 22

possible, probable w/ lab support , probable and definited

Question 23

definite als

Answer 23

UMN/LMN in 3 regions or more

Question 24

probable als

Answer 24

UMN in 2 region with some UMN above LMN

Question 25

probable with lab support

Answer 25

UMN/LMN in 1 region with 2 regions with LMN

Question 26

possible

Answer 26

umn/lmn in 1 region or , isolated UMN in 2 or more, or LMN rostral to UMN

Question 27

CK elevation in ALS

Answer 27

usually less than 1k

Question 28

DDX ALS: LMN mimics

Answer 28

benign fascic, IBM, MMN, NA, Monomelic amyotrophy, SBMA, CMT/dSMA, post-polio syndrome

Question 29

DDX ALS: UMN/LMN mimic

Answer 29

polyradiculomyelopathy

Question 30

DDX ALS: UMN mimics

Answer 30

nutritional, HSP, adrenomyeloneuropathy, late onset Tay-Sachs , polyglucosan body, HIV myelopathy, MS

Question 31

SBMA diagnostic clues

Answer 31

slow progression, facial twitching, tremor , sensory neuropathy, androgen insensitivity

Question 32

MMN vs ALS

Answer 32

nerve rather myotomal pattern anti-gm1 anitbodies

Question 33

adrenomyeloneuropathy diagnostic clues

Answer 33

sensory neuropathy, w/w/o adrenal insufficiency, x- linked (ABCD1)

Question 34

late onset tay-sachs diagnostic clues

Answer 34

cerebellar ataxia, psych, ashkenazi HEXA

Question 35

polyglucosan body disease

Answer 35

distal sensory loss, neurogenic bladder cerebellar ataxia, cognitive deficits.

Question 36

PLS remains isolated to UMN for

Answer 36

4 years usually

Question 37

PMA to ALS

Answer 37

20% by 5 years