Abstract

1. Clinical backgroundA 58-year-old man presented with a four-year history ofgradually increasing gait unsteadiness, urinary inconti-nence and erectile dysfunction. His daughter complainedthat he snored loudly at night, but on further questioningit was apparent that this was an inspiratory stridor. Therewas no other past history, and no family history of note.Examination showed gaze-evoked nystagmus, mild dysar-thria, and severe truncal and limb ataxia. Blood pressuredid not fall on standing. An MRI brain was performed(Figs. 1, 2).2. What is the most likely diagnosis?1. Idiopathic late onset cerebellar ataxia.2. Olivo-ponto-cerebellar atrophy variant of multiple sys-tem atrophy.3. Spino-cerebellar ataxia.4. Gluten ataxia.Answer on page 307.

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