Module 13

Question 1

What is the definition of Amyotrophic Lateral Sclerosis?

Answer 1

ALS, also known as Lou Gehrig’s Disease, is a degenerative disease that affects both UMN’s and LMN’s and is the most common type of motor neuron disease (MND)

Question 2

What is the genetic influence of ALS?

Answer 2

sporadic ALS 90-95%

familial ALS 5-10%

Question 3

What is the gender influence of ALS?

Answer 3

Males 1.7:1

Question 4

What is the average range of age of onset of ALS?

Answer 4

55-60 years

Question 5

What is the prevalence rate of ALS?

Answer 5

30,000

Question 6

What is the incidence rate of ALS?

Answer 6

5,000

Question 7

What is the mortality rate of ALS?

Answer 7

3-5 years from onset

Question 8

What is the cause of ALS?

Answer 8

Occur as the result of an intersection of a variety of cellular insults, such as superoxide dismutases, leading to neuron degeneration and death.
Research suggests an excess of glutamate in the synaptic cleft triggers a cascade of events that leads to cellular death.

Question 9

What is the pathophysiology of ALS?

Answer 9

progressive motor neuron loss in spinal cord, brainstem, and cortex.
partial sparing of cranial nerves III, IV, and VI, sacral spinal level, spinocerebellar tract, smooth muscle, cardiac muscle, sensory tracts, and cognition

Question 10

What is the progression of independence of ALS?

Answer 10

early stage: independent
middle stage: partially independent
late stage: dependent

Question 11

What is the diagnosis criteria of ALS?

Answer 11

objective evidence of UMN and LMN damage
minimum of 3 regions affected
disease progression

Question 12

What disease modifying drugs are used in ALS?

Answer 12

Rilutek

Question 13

What symptomatic drugs are used in ALS?

Answer 13

Pain-Vicodin
Fatigue- Amantadine
Spasticity- Baclofen
Sialorrhea- Artane
Depression- Paxil