A 67 year old woman with a two year history of laryngopharyngeal dystonia, spasmodic dysphonia, and parkinsonism succumbed to Wernicke's encephalopathy and died six months later. Necropsy showed, besides Wernicke's encephalopathy, degenerative changes in selected thalamic nuclei (dorsomedial, pulvinar, and the medial geniculate bodies) and the inferior olives and numerous cerebellar torpedoes. The substantia nigra and basal ganglia were spared. Immunostaining for prion protein was negative. This patient indicated a new type of presentation of so-called pure thalamic degeneration, or more precisely thalamo-olivary degeneration.
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