Amyotrophic Lateral Sclerosis

Question 1

In a subset of cases, ALS arises concurrently with…?

Answer 1

Frontotemporal Dementia.

Question 2

Degeneration of corticobulbar projections innervating the brainstem results in…?

Answer 2

Dysarthria and exaggeration of the motor expressions of emotion. The latter leads to involuntary excess in weeping or laughing (pseudobulbar affect)

Question 3

When is ALS marked as definite ?

Answer 3

When three or four of the following are involved : bulbar, cervical, thoracic, and lumbosacral motor neurons

Question 4

3 findings which distinguish X-Linked Spinobulbar Muscular Atrophy (Kennedy’s Disease) from ALS ?

Answer 4

Gynecomastia, Absence of pyramidal tract disease (spasticity) and the presence of a subtle sensory neuropathy in some patients.