Neuromuscular and nervous systems pathology- Amyotrophic Lateral Sclerosis (ALS)

Question 1

what kind of disease is ALS?

Answer 1

a chronic degenerative disease that produces both upper and lower motor neuron impairments

Question 2

ALS produces both __ and __ motor neuron impairments

Answer 2

upper and lower

Question 3

what is the pathology of ALS disease?

Answer 3

significant loss of anterior horn cells in the spinal cord and motor cranial nerve nuclei in the lower brainstem produce weakness and muscle atrophy
demyelination of corticospinal and corticobulbar tracts produce UMN symptoms

Question 4

the pathology of ALS includes significant loss of __ __ __ in the spinal cord and __ __ __ __ in the __ brainstem and produces weakness and muscle atrophy

Answer 4

anterior horn cells; motor cranial nerve nuclei; lower

Question 5

the pathology of ALS includes significant loss of anterior horn cells in the spinal cord motor cranial nerve nuclei in the lower brainstem and produces __ and __ __

Answer 5

weakness and muscle atrophy

Question 6

the pathology of ALS includes demyelination of the __ and __ tracts producing UMN symptoms

Answer 6

corticospinal and corticobulbar tracts

Question 7

the pathology of ALS includes demyelination of the corticobulbar and corticospinal tracts producing __ __ __ symptoms

Answer 7

UMN

Question 8

what symptoms (3) does the rapid degeneration associated with ALS cause?

Answer 8

denervation of muscle fibers
muscle atrophy
muscle weakness

Question 9

is there an exact known etiology of ALS?

Answer 9

no

Question 10

what are the hypothesized causes of ALS?

Answer 10

genetic inheritance virus
metabolic disturbances
lead and aluminum toxicity

Question 11

there is a higher incidence of ALS in men/women and the disease typically begins between __-__ years of age

Answer 11

men; 40-70

Question 12

early clinical presentation/signs and symptoms (8) of ALS?

Answer 12

upper and lower motor neuron involvement:

LMN-
asymmetric muscle weakness fasciculations
cramping
atrophy within hands

UMN-
incoordination of movement
spasticity
clonus
positive babinski refelx

Question 13

as the disease progresses, what signs and symptoms will a patient with ALS eventually begin demonstrating (3)?

Answer 13

oral motor impairment
motor paralysis
eventual respiratory distress

Question 14

what UMN symptoms (4) present in a patient with early stage ALS disease?

Answer 14

incoordination of movement
spasticity
clonus
positive babinski reflex

Question 15

what LMN symptoms (4) present in a patient with early stage ALS disease?

Answer 15

asymmetric muscle weakness
fasciculations
cramping
muscle atrophy in hands

Question 16

weakness in ALS spreads in a __ to __ pattern?

Answer 16

distal to proximal

Question 17

effective management of ALS is based on what two factors?

Answer 17

supportive care
symptomatic therapy

Question 18

pharmacological management of ALS?

Answer 18

riluzole (Rilutek)

Question 19

what 5 fields of therapies may be warranted for a patient with ALS? what should these therapies be focused on (2)?

Answer 19

PT, OT, SLP, nutrition, respiratory
quality of life and caregiver training