All issues > Volume 49(2); 2006

Case Report

Korean J Pediatr. 2006;49(2):208-211. Published online February 15, 2006.

A case of Dyke-Davidoff-Masson syndrome in Korea

Jun Hwa JH Lee¹, Zee Ihn ZI Lee², Ho Kyun HK Kim³, Soon Hak SH 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

Correspondence Soon Hak SH 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.

Keywords :Dyke-Davidoff-Masson Syndrome , Hemiatrophy , Hemiplegia , Seizure