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Images in Neurology |

Progressive Ataxia and Palatal Tremor

José Rafael P. Zuzuárregui, MD1; Samuel A. Frank, MD1
[+] Author Affiliations
1Department of Neurology, Boston University School of Medicine, Boston, Massachusetts
JAMA Neurol. 2015;72(10):1195. doi:10.1001/jamaneurol.2015.1114.
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Extract

This case report describes ataxia with uncontrollable palatal tremor in an elderly man.

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.

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Figure.

Axial T2-weighted magnetic resonance imaging of the brain and brainstem without contrast demonstrates significant cerebellar atrophy.

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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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Video.

Spontaneous palatal myoclonus. The patient was unable to stop myoclonus of the palate with voluntary use of the palatal musculature. No clicking was noted by the patient or examiners.

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