A 56-year-old African American man was evaluated for intermittent muscle weakness, resting tremor, unsteady gait, and frequent falls. The onset of the symptoms occurred 10 years before the evaluation and was followed by a slow deterioration. At 25 years of age, the patient had 2 surgical procedures for removal of disfiguring accumulations of fat around the anterior and posterior of the neck. His medical history was otherwise unremarkable, and there was no history of alcohol abuse, diabetes mellitus, thyroid diseases, and dyslipidemia. His family history was significant for a sister who was diagnosed with multiple sclerosis, neuropathy, and narcolepsy. His mother, maternal grandmother, and maternal aunt were reported to have lipomas, but none of these individuals were known to have additional neuromuscular problems.