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A Rare Manifestation of Familial Cavernous Angiomas

Sudhir Malik, MD; Bruce H. Cohen, MD; John Robinson, MD; Arno Fried, MD; Cathy A. Sila, MD

Arch Neurol. 1992;49(2):170-173.

Abstract
• We studied four generations of a family in which the index case had progressive loss of vision secondary to a cavernous angioma of the optic nerve and chiasm. Magnetic resonance imaging of the brain revealed multiple, asymptomatic intracerebral cavernous angiomas. Brain magnetic resonance imaging scans of the family members revealed multiple cavernous angiomas in the brother and paternal grandfather, but none in the father or his siblings. Autopsy reports of the paternal great grandfather noted multiple cavernous angiomas in the brain and abdominal viscera. We believe our patient to be the sixth reported case in which a cavernous angioma involved the optic chiasm and optic nerve. Magnetic resonance imaging is a sensitive and specific method of detecting cavernous angiomas. Cavernous angiomas have an autosomal dominant pattern of inheritance with variable penetrance. Surgical intervention in patients with symptomatic cavernous angiomas depends on the location and size of the lesion and associated surgical risks.

Author Affiliations
From the Departments of Neurology (Drs Malik, Cohen, and Sila) and Neurosurgery (Drs Robinson and Fried), Cleveland (Ohio) Clinic Foundation.

Footnotes
Accepted for publication September 3, 1991.

Presented as a poster at the 19th Annual Child Neurology Society Meeting, Atlanta, Ga, October 18,1990.

Reprint requests to the Department of Neurology, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195 (Dr Cohen).

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