ALS

Question 1

ALS

Answer 1

amyotrophic lateral sclerosis

Question 2

ALS is also known as

Answer 2

lou gehrigs dz

Question 3

what is ALS

Answer 3

most common form of adult onset progressive motor neuron dz

may be the most devastating of the neurologic degenerative dz

Question 4

hallmark of ALS

Answer 4

combo of UMN and LMN signs and symptoms

Question 5

UMN signs

Answer 5

spasticity

hyperreflexia

pathological reflexes

Question 6

where do UMN signs stem from

Answer 6

destruction of motor neurons int he brain

causing degeneration of the corticospinal and corticobulbar tracts

Question 7

LMN signs

Answer 7

muscle wasting

weakness

fasciculations

Question 8

muscle weakness

Answer 8

amyotrophy

Question 9

LMN signs stem from

Answer 9

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

Question 10

90% of the CST fibers

Answer 10

decussate at the medulla

descend in the SC as the lateral CST

Question 11

lateral sclerosis

Answer 11

destruction and scarring of the lateral CST

Question 12

classification

Answer 12

sporadic

familial

Question 13

sporadic

Answer 13

the most common form of ALS in the US

90-95% of all cases

Question 14

familial

Answer 14

occurring more than once in family lineage (autosomal dominant)

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

Question 15

familial is caused by

Answer 15

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

Question 16

initial S&S

Answer 16

limb onset ALS

bulbar onset ALS

Question 17

limb onset ALS

Answer 17

75% of cases

Question 18

limb onset ALS is

Answer 18

difficulty walking or difficulty with the fine motor coordination of UEs

Question 19

bulbar onset ALS

Answer 19

25% of cases

Question 20

bulbar onset is

Answer 20

dysphagia

dysarthria

overactive gag reflex

Question 21

what motor nuclei are involved in bulbar

Answer 21

CN 5,7,9,10,12

Question 22

weakness and atrophy –> bulbar onset

Answer 22

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

Question 23

what CN are typically spared –> bulbar onset

Answer 23

3,4,6

Question 24

pseudobulbar affects

Answer 24

15-45% of bulbar onset

Question 25

3 stages of diagnosis

Answer 25

clinically possible clinically probable clinically definite

Question 26

clinically possible

Answer 26

UMN and LMN findings in the same region

Question 27

clinically probable

Answer 27

UMN and LMN findings in 2 regions

Question 28

clinically definite

Answer 28

UMN and LMN findings in 3 or more regions

Question 29

regions

Answer 29

bulbar cervical thoracic lumbosacral

Question 30

dx is supported by

Answer 30

spread of signs and symptoms from one region to another with a linear increase in severity

Question 31

dx can be ruled out by

Answer 31

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

Question 32

how to dx

Answer 32

exclusion no definite tests for it

Question 33

exclusion tests

Answer 33

CSF CT/MRI biopsy or ms or nerve lab tests

Question 34

other motor neuron dz

Answer 34

spinal muscle atrophy primary lateral sclerosis

Question 35

spinal muscle atrophy

Answer 35

autosomal recessive genetic LMN dz of childhood

Question 36

primary lateral sclerosis

Answer 36

slowly progressive UMN dz characterized by spasticity and hyperreflexia

Question 37

onset of primary lateral sclerosis

Answer 37

after 40

Question 38

is PLS an subset of ALS

Answer 38

unknown

Question 39

incidence

Answer 39

4-6/100000

Question 40

incidence gender

Answer 40

men > women by 2:1 except bulbar onset affects females and older people more

Question 41

90% of ALS occurs

Answer 41

sporadically with an unknown cause

Question 42

mean age at onset

Answer 42

57

Question 43

clinical presentation

Answer 43

no lab test to confirm dx nerve conduction velocity is normal emg consistent with LMN dz

Question 44

dz is based on

Answer 44

recognition of clinical pattern or syndrome one of exclusion

Question 45

first and earliest symptoms notes in 90% of pt

Answer 45

striated muscle weakness initially focal but grows to include the entire body

Question 46

onset is

Answer 46

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

Question 47

physical exam

Answer 47

MMT will show greater weakness than the pt reports

Question 48

what occurs by the time pt c/o weakness

Answer 48

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