A man in his 70s presented with a 20-year history of progressive ataxia and an 8-month history of “spasms” in his throat. He was initially believed to have cerebellar degeneration due to alcohol abuse. However, his difficulty with gait progressed after cessation of alcohol use. The reported throat spasms did not affect his ability to speak or eat. Examination revealed hypometric saccades, truncal and limb ataxia, and decreased vibratory sense with an inability to ambulate. Bradykinesia and mild rigidity were also noted. Palatal myoclonus was seen at a frequency of 1 to 2 Hz (Video). Severe cerebral and cerebellar atrophy was demonstrated on magnetic resonance imaging of the brain (Figure). Results of testing for spinocerebellar ataxia types 1, 2, 3, 6, and 12 were normal.
Zuzuárregui JRP, Frank SA. Progressive Ataxia and Palatal Tremor. JAMA Neurol. 2015;72(10):1195. doi:10.1001/jamaneurol.2015.1114