ALS

Question 1

most common adult-onset motor neuron disease:

Answer 1

ALS

Question 2

commonly known as _______ disease

Answer 2

Lou Gehrig’s

Question 3

ALS means:

Answer 3

amyotrophic: no muscle
lateral: spinal cord where motor neurons are located
sclerosis: hardening of the affected muscles

Question 4

two classifications of ALS:

Answer 4

familial ALS: family hx –> mostly autosomal dominant
sporadic ALS: no family hx

Question 5

______% develop limb onset ALS
______% develop bulbar onset ALS
regardless of initial involvement, both ______ and ____ are implicated in the disease

Answer 5

70-80%
20-30%
UMN;LMN

Question 6

ALS is always _______ and with disease progression individual will become ______ in all aspects of life

Answer 6

fatal; dependent

Question 7

Epidemiology: higher incidence in men or women?
average age of onset?

Answer 7

men; 50s

Question 8

Etiology is largely _______
Possible causes:
excess ______ causing ecotoxicity
____ mutation
inflammation/autoimmune response
growth factor abnormalities
viral infections
apoptosis (programmed cell death)

Answer 8

unknown
glutamate
SOD - 1

Question 9

known risk facks of ALS:

Answer 9

age
gender
family hx
clusters (ex. western pacific ALS/PDC)
disease-causing mutations

Question 10

unknown risk factors for ALS:

Answer 10

diet
vigorous physical activity
trauma
neurotoxicant exposure
lifestyle factors

Question 11

ALS involves the loss of ______ neurons in the _______ cortex, _____ and ______
-typically ALS progresses ________ a body region before moving to a neighboring region of the body
-_____ onset ALS may spread to the cervical region

Answer 11

motor; motor, spinal cord, brainstem
within/single
bulbar

Question 12

CN commonly affected includes:

Answer 12

CN V, VII, IX, X, XII

Question 13

ALS involves degeneration and eventual loss of motor neurons, but early on reinnervation from healthy axons to denervated muscles. With disease progression:
—______occurs

Answer 13

reinnervation cannot keep up with the rate of denervation
muscle atrophy

Question 14

ALS does not just affect motor areas but nonmotor such as

Answer 14

ANS
basal ganglia
cerebellum
frontotemporal
oculomotor
sensory systems

Question 15

Clinical manifestations in ALS:

Answer 15

extent of motor neuron loss
combination of UMN and LMN loss
non-motor area affected
pattern of onset
body regions affected
sec. complication
stage of disease

Question 16

LMN Signs:

Answer 16

muscle weakness
hyporeflexia
hypotonicity
muscle cramps
atrophy
fasciculations

Question 17

UMN signs

Answer 17

spasticity
hyperreflexia
pathologic reflexes
muscle weakness

Question 18

bulbar pathology:
spastic bulbar palsy (____)
flaccid bulbar palsy (____)
is mixed palsy common?

Clinical presentation: (5)

Answer 18

UMN
LMN
yes

bulbar muscle weakness
dysphagia
dysarthria
sialorrhea
pseudobulbar affect

Question 19

What is the leading cause of death in individuals with ALS?
pt. complaints: (5)

Answer 19

respiratory failure

fatigue
decrease activity level
inability to tolerate supine
headache due to hypoxia
changed in speech

Question 20

cognitive deficits may include:

Answer 20

decreased language comprehension
impaired memory
difficulty w/abstrct reasoning
frontotemporal dementiaia

Question 21

bulbar onset is more predictive of ____ impairment than limb onset

Answer 21

cognitive

Question 22

clinical manifestations: rare (3)

Answer 22

sensory
bowel and bladder
ocular palsy

Question 23

there is no single test to diagnose someone with ALS
a diagnosis of ALS is made by ______ of disease progression and testing to rule out other neurologic diseases
tests to rule out: (6)

Answer 23

observation
EMG, lab studies, neuroimaging, nerve conduction testing, and/or muscle and nerve biopsies

Question 24

symptom onset to diagnosis can range average from _____-____ months
diagnostic criteria to aid physicians in the diagnosis of ALS (2)

Answer 24

8-15 months
el escorial criteria
Awaji-shima criteria