Dyke-Davidoff-Masson Syndrome: Cerebral Hemiatrophy - Five cases with Review of literature

Abstract

Introduction-Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome (DDMS) is uncommon entity in pediatric age group. It is characterized clinically by hemiparesis, seizures, mental retardation and has hallmark radiological picture which includes hemiatrophy of the cerebral hemisphere with hypertrophy of ipsilateral skull vault, hyerpneumatization of paranasal sinuses, mastoid air cells and elevation of petrous ridge. Most of the published data about this syndrome is of case reports. We report series of five pediatric cases from central India of cerebral hemiatrophy with classical clinical and radiological findings with short review of literature.
Case report- We have reported five cases of DDMS over a period of two years between July 2010 to August 2012 in pediatric age group who reported to us for imaging. Age of the patient varied between 5 months to 8 years which included one female child and four male children. Presenting feature were hemiplegia, mental retardation, seizures. On imaging cerebral atrophy contralateral to the side of hemiplegia is seen with calvarial thickening.
Conclusion- DDMS is characterized by triad of hemiparesis, contralateral cerebral hemiatrophy and seizures along with classical radiological features. Imaging has distinguished role in diagnosing this rare entity and differentiating it from other causes of cerebral hemiatrophy.