Familial cavernous angiomas manifesting with an acute chiasmal syndrome.
We studied a family in which the proband had an acute chiasmal syndrome secondary to a cavernous angioma of the optic nerve and chiasm. A brain magnetic resonance imaging scan indicated additional asymptomatic intracerebral cavernous angioma, and several of the patient's relatives were found to have various central nervous system vascular malformations. Magnetic resonance imaging may distinguish, noninvasively, symptomatic and asymptomatic carriers of cavernous angiomas and further delineate their inheritance patterns. Asymptomatic cavernous angiomas of the central nervous system are best followed without intervention, whereas symptomatic patients should be treated on an individual basis, depending on the location of lesion and the risk of surgical removal.
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia.
J R
JR
Corboy
S L
SL
Galetta
D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis; D002648:Child; D003390:Cranial Nerve Neoplasms / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D006392:Hemangioma, Cavernous / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis; D009897:Optic Chiasm; D009900:Optic Nerve; D013120:Spinal Cord Neoplasms / Q000175:diagnosis