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Brain SPECT Evaluation of Cerebral Perfusion in Hemimegalencephaly

Hemimegalencephaly is a rare congenital neuronal migration disorder characterized by unilateral hypertrophy of a cerebral hemisphere. It can be an isolated abnormality, or it can be associated with neurocutaneous syndromes. Clinical manifestations include intractable motor seizures, impaired psychom...

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Bibliographic Details
Published in:Clinical nuclear medicine 1997-04, Vol.22 (4), p.250-252
Main Authors: BAR-SEVER, ZVI, CONNOLLY, LEONARD P, BARNES, PATRICK D, TREVES, S TED
Format: Article
Language:English
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Summary:Hemimegalencephaly is a rare congenital neuronal migration disorder characterized by unilateral hypertrophy of a cerebral hemisphere. It can be an isolated abnormality, or it can be associated with neurocutaneous syndromes. Clinical manifestations include intractable motor seizures, impaired psychomotor development, hemiparesis, and hemianopsia. Prognosis is poor. Hemispherectomy is the treatment of choice. Brain SPECT is useful in excluding seizure foci or other perfusion abnormalities in the contralateral side before surgery. The authors report two cases of increased perfusion in the affected hemisphere relative to the contralateral hemisphere that contrast with previous reports of decreased perfusion in the abnormal side as determined scintigraphically.
ISSN:0363-9762
1536-0229
DOI:10.1097/00003072-199704000-00009