Amyotrophic Lateral Sclerosis or "Lou Gehrig Disease"

Question 1

ALS disorder affects…

Answer 1

Anterior Horn Cells and corticospinal tracts

+Corticobulbar involvement…

Presence of UMN and LMN signs = hallmark of ALS

Only MOTOR system is involved

Question 2

Clinical Features

Answer 2

• Progressive muscle weakness = hallmark
• Muscle cramps/spasticity
• Fasciculations
• Impaired speech and swallowing; dysphagia –> aspiration
• Respiratory muscle weakness –> dyspnea on exertion, and later at rest
• Weight loss and fatigue

Question 3

What clinical features are NORMAL and UNAFFECTED

Answer 3

• Bladder and bowel control
• Sensation
• Cognitive fxn
• Extraocular muscles
• Sexual fxn

Question 4

Dx

Answer 4

No specific dx test for ALS

EMG and nerve conduction studies can confirm degeneration of LMNs and r/o NMJ disorders

Question 5

Tx

Answer 5

… been very disappointing –> mainly supportive

RILUZOLE = glutamate-blocking agent–may delay death by only 3 to 5 months