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Clinicopathologic Report
February 1999

Neuro-ophthalmic, Radiographic, and Pathologic Manifestations of Adult-Onset Alexander Disease

Author Affiliations

From the Departments of Ophthalmology (Drs Martidis and Yee), Pathology (Dr Azzarelli), and Neurology (Dr Biller), Indiana University School of Medicine, Indianapolis.

 

W. RICHARDGREENMD

Arch Ophthalmol. 1999;117(2):265-267. doi:10.1001/archopht.117.2.265
Abstract

A 61-year-old woman had a 3-year history of imbalance. Eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles and dentate nuclei, which enhanced with gadolinium. Histopathological analysis of a needle biopsy specimen of the left cerebellar peduncle revealed diffuse gliosis in the presence of symmetrically distributed areas of demyelination. There were associated Rosenthal fibers. Clinicopathologic correlation supported a diagnosis of Alexander disease. An adult patient with a history of progressive imbalance, ocular motility abnormalities consistent with cerebellar and/or brainstem dysfunction, and diffuse, symmetric hyperintense magnetic resonance imaging signals in brainstem and cerebellar white matter should suggest a diagnosis of adult-onset Alexander disease.

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