ALS

Question 1

what is the most common and fatal motor neuron disease

Answer 1

ALS

Question 2

mortality rate

Answer 2

2-5 years
variable

Question 3

what is ALS characterized by

Answer 3

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

Question 4

what is specifically degenerated

Answer 4

anterior horn cells
corticobulbar tracts
corticospinal tracts

Question 5

what is onset

Answer 5

> 50 years

Question 6

men or women are more affected

Answer 6

men, slightly

Question 7

what causes the UMN symptoms

Answer 7

degeneration of UMN cell bodies in motor cortex

Question 8

what causes the LMN symptoms

Answer 8

alpha motor neurons LMN of spinal cord

Question 9

what is one cause of ALS

Answer 9

glutamate excitotoxicity

Question 10

what does glutamate excitotoxicity increase

Answer 10

increased CSF, plasma, post-mortem tissue

Question 11

risk factors for ALS

Answer 11

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

Question 12

diagnosis of ALS

Answer 12

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

Question 13

what does a clinical dx of ALS require

Answer 13

pattern of observed and reported symptoms of both UMN/LMN

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

Question 14

what is the El escorial criteria for dx of ALS

Answer 14

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

Question 15

what regions are involved with El escorial criteria

Answer 15

defined as bulbar, cervical, thoracic, lumbosacral

Question 16

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

Answer 16

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

Question 17

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

Answer 17

bulbar, UE/LE

Question 18

what does El escorial criteria dx lead to

Answer 18

suspected, possible, probable and definite ALS

Question 19

what does gold coast criteria for dx lead to

Answer 19

+ ALS or - ALS

Question 20

cardinal sign of ALS

Answer 20

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

Question 21

do you see more UMN weakness or LMN

Answer 21

LMN weakness

Question 22

what happens to eye, bowel and bladder

Answer 22

normal spared until terminal stages

Question 23

what do respiratory impairments look like for this population

Answer 23

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

Question 24

what do cognitive impairments look like for this population

Answer 24

variable
verbal fluency, language comprehension, memory, abstract reasoning

Question 25

who are cognitive impairments more common in

Answer 25

bulbar onset

Question 26

additional impairments

Answer 26

pain
fatigue
ANS symptoms

Question 27

what is spared in this disease

Answer 27

sensory pathways
ocular motor fxn
sphincter (bowel/bladder)