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A case of Dyke-Davidoff-Masson syndrome in Korea

Korean Journal of Pediatrics 2006;49(2):208-211.
Published online February 15, 2006.
A case of Dyke-Davidoff-Masson syndrome in Korea
Jun Hwa Lee1, Zee Ihn Lee2, Ho Kyun Kim3, Soon Hak Kwon4
1Department of Pediatrics, Catholic University of Daegu, School of Medicine
2Department of Physical Medicine & Rehibilitation Catholic University of Daegu, School of Medicine
3Department of Radiology, Catholic University of Daegu, School of Medicine
4Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea
국소적 경련과 편마비를 동반한 Dyke-Davidoff-Masson 증후군 1례
이준화1, 이지인2, 김호균3, 권순학4
1대구가톨릭대학교 의과대학 소아과학교실
2대구가톨릭대학교 의과대학 재활의학교실
3대구가톨릭대학교 의과대학 방사선과학교실
4경북대학교 의과대학 소아과학교실
Correspondence: 
Soon Hak Kwon, Email: shkwon@knu.ac.kr
Abstract
Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.
Key Words: Dyke-Davidoff-Masson Syndrome , Hemiatrophy , Hemiplegia , Seizure


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