Teaching Video NeuroImages: Optic Ataxia as the Presenting Sign of the Heidenhain Variant of Creutzfeldt-Jakob Disease

A 59-year-old woman presented with subacute onset of hand clumsiness followed by memory loss. She reported difficulty reaching for objects, although she could see them. Examination (video) showed optic ataxia, visual acuity of 20/30 OU, intact visual field, and near-normal finger-to-nose test. Hyperintense signals in the left temporo-parieto-occipital cortex in diffusion-weighted MRI, periodic triphasic complexes in EEG (figure), and elevated t-tau in CSF suggested the Heidenhain variant of Creutzfeldt-Jakob disease.1 The patient received supportive care and progressed to akinetic mutism. Optic ataxia—inaccuracy of visually guided arm movements—is a visuomotor disorder associated with lesions involving the posterior parietal cortex.2

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