Amyotrophic Lateral Sclerosis (ALS)

Question 1

ALS (“Lou Gehrig’s disease”)

Answer 1

Neurodegenerative movement disorder
Causes progressive loss of motor neurons within the spinal cord and cranial nerves
Results in continual difficulties with controlling voluntary movements
Initial clinical presentation can be classified as limb-onset ALS (about 70%) or bulbar-onset ALS (about 25%)
Disease later spreads to other regions of the body

Question 2

Early symptoms

Answer 2

Limb onset - muscle twitching, cramps, weakness, stiffness
Bulbar onset - muscle twitches in tongue, slurred/nasal speech, difficulties chewing and swallowing

Question 3

Epidemiology

Answer 3

Onset usually between 58-60
Onset before age 30 occurs in less than 5% of patients
Average survival from onset to death is 3-4 years
Juvenile onset ALS is associated with a shorter survival year (average 1.9 years)

Question 4

Cognitive and behavioral features of ALS

Answer 4

35-50% of patients with ALS exhibit impairments in non-motor mediated cognitive functioning (executive dysfunction often seen first)
Symptoms of depression, apathy, and low motivation are observed in greater than 25% of patients