The Proteus Syndrome: CNS Imaging Findings

The Proteus Syndrome: CNS Imaging Findings

The Proteus syndrome is a complex disorder characterised by a constellation of hamartomatous, mesodermal and skeletal manifestations. Major clinical findings include hemihypertrophy partial gigantism of the hands and/or feet, skin pigmentation and subcutaneous/osseous tumours. CNS manifestations of...

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Bibliographic Details
Published in:Rivista di neuroradiologia Vol. 12; no. 4; pp. 575 - 579
Main Authors: Jhaveri, K.S., Shetty, RG, Shroff, M.M., Kamat, N., Bavadekar, M., Udani, V.P.
Format: Journal Article
Language:English
Published: 01-08-1999
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Summary:The Proteus syndrome is a complex disorder characterised by a constellation of hamartomatous, mesodermal and skeletal manifestations. Major clinical findings include hemihypertrophy partial gigantism of the hands and/or feet, skin pigmentation and subcutaneous/osseous tumours. CNS manifestations of hemimegalencephaly subependymal calcified nodules, periventricular cysts and dural sinus thrombosis have been reported. We report a case of proteus syndrome in which abnormal hyperintense signal was seen in the globus pallidi and posterior limbs of internal capsule on T1 weighted image on MR in addition to cortical dysplasia, macrocephaly, ventriculomegaly and abnormal periventricular white matter.
ISSN:1120-9976
DOI:10.1177/197140099901200412