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Dyke Davidoff Masson Syndrome – A Case Report

机译：Dyke Davidoff Masson综合征–病例报告

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A 10 year old male child reported to paediatric neurology OPD of BIRDEM General Hospital with the complaints of weakness of left side of body, seizure and facial asymmetry for 2 years. He was on regular anti convulsive therapy and failed to control seizure. General examination revealed no significant abnormality neither delayed mile stones of development happened. Neurological examination revealed left sided spastic hemiparesis, brisk tendon reflexes and extensor planter on left side. With detailed history and examination he was diagnosed as a case of infantile seizure and undergone CT scan of brain followed by MRI scan. Both the reports revealed severe atrophy of right cerebral hemisphere, thinning of cortical gyri, widening of sulci and dilatation of right lateral ventricle with ipsilateral midline shift and was concluded as hemiatrophy of right cerebral hemisphere with suspicion of Dyke Davidoff Masson Syndrome (DDMS), Hemimegalencephaly and Sturge-Weber syndrome. Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. Considering clinical history and imaging findings, final Diagnosis was Dyke Davidoff Masson Syndrome. Birdem Med J 2013; 3(2): 196-109 DOI: http://dx.doi.org/10.3329/birdem.v3i2.17215
机译：一个10岁的男孩向BIRDEM总医院的小儿神经病学OPD报告了2年的症状，他们抱怨身体左侧无力，癫痫发作和面部不对称。他接受常规抗惊厥治疗，未能控制癫痫发作。常规检查未发现任何明显异常，也没有发生发育迟缓的里程碑。神经系统检查显示左侧痉挛性偏瘫，左侧轻度肌腱反射和伸肌种植体。根据详细的病史和检查，他被诊断为婴儿癫痫发作，并先后进行了脑部CT扫描和MRI扫描。两项报告均显示右脑半球严重萎缩，皮质回旋变薄，右脑室沟扩大和同侧中线移位，并得出结论为右脑半球性贫血，并怀疑戴维夫·大卫森·马森综合征（DDMS），半巨脑和Sturge-Weber综合征。 Dyke-Davidoff-Masson综合征（DDMS）是一种罕见的疾病，其特征在于大脑半球不对称生长，具有单侧萎缩，同侧代偿性骨肥大，鼻旁窦和乳突细胞过度气化以及对侧轻瘫。 DDMS可能导致不同程度的偏瘫，偏瘫，癫痫发作，智力低下和面部不对称。考虑到临床病史和影像学发现，最终诊断为戴维·大卫杜夫·马森综合征。 Birdem Med J 2013; 3（2）：196-109 DOI：http：//dx.doi.org/10.3329/birdem.v3i2.17215

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