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Neurology 1999;53:629
© 1999 American Academy of Neurology
Brief Communications

Cerebroretinal vasculopathy mimicking a brain tumor: A case of a rare hereditary syndrome

S. Weil, MD, G. Reifenberger, MD, PhD, C. Dudel, MD, T. A. Yousry, MD, S. Schriever, MD and S. Noachtar, MD

From the Departments of Neurology (Drs. Weil, Dudel, and Noachtar), Neuroradiology (Dr. Yousry), and Ophthalmology (Dr. Schriever), Ludwig-Maximilians-University, Munich, Germany; and the Department of Neuropathology (Dr. Reifenberger), University of Bonn Medical Center, Germany.

Address correspondence and reprint requests to Dr. Sabine Weil, Department of Neurology, Ludwig-Maximilians-University, Klinikum Großhadern, Marchioninistr.15, D-81337 Munich, Germany.

We report a 35-year-old man with hereditary cerebroretinal vasculopathy (CRV) characterized by retinal microvascular changes and a right frontal intracerebral mass lesion that suggested a brain tumor. Histopathologic analysis of the patient’s brain lesion as well as reviewed specimens of the patient’s mother, who had reportedly died of a brain tumor, showed no neoplasia but did show cerebral microvasculopathy. CRV should be considered as a differential diagnosis for patients with intracerebral mass lesions, retinal vascular changes, and a positive family history of "brain tumors."




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