High-definition spectral domain optical coherence tomography in the evaluation of ataxia with visual impairment.

摘要

A 36-year-old man was evaluated for a 10-year history of visual decline and 5-year history of gait, limb, and speech ataxia. His neurologic exam was remarkable for a wide based ataxic gait with appendicular, axial and speech ataxia. His eye exam revealed saccadic pursuits and reduced visual acuity. Ophthal-moscopic evaluation of the fundus was remarkable for diffuse attenuation of the arterioles and minimal foveal fine pigmentary mottling. A full-field electroretinogram revealed abnormalities that tended to be more prominent in cone-mediated responses. Visual field assessment showed increased blind spot and a left pericentral scotoma. Fluorescein angiography showed a small central window defect in the macula (figure 1A).