Abstract
A 19 years old female presented with recurrent generalized seizures, right sided hemiplegia, and had developmental delay in motor and speech domains. CT of the brain revealed characteristic features diagnostic of infantile type of cerebral hemi atrophy or Dyke-Davidoff-Masson syndrome. It is an important cause of intractable and drug-resistant seizures. It has varied clinical presentation and history with distinct neuroimaging features. Here, we describe a female patient presented with recurrent intractable convulsion, mental retardation, hemiparesis, dystonia and characteristic neuroimaging features of cerebral hemiatrophy, calvarial thickening, and ipsilateral hyperpneumatization of the frontal sinuses which is suggestive of DDMS. Early institution of neuroimaging in patients with intractable epilepsy will make early diagnosis and better outcome. DDMS also occur due to atrophy or hypoplasia of one cerebral hemisphere (hemiatrophy), which is usually due to any trauma or injury to the growing brain in early childhood. The manifestations are variable and depend on the extent of brain injury. More common symptoms are recurrent seizures, facial asymmetry, hemiplegia, mental retardation, mutism. The CT of brain mostly give impression of cerebral hemiatrophy with ipsilateral hypertrophy of the skull and sinuses.
Keywords: Dyke-Davidoff-Massion Syndrome, hemiparesis, mental retardation, hemiatrophy, hyperpneumatization.
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Corresponding Author
Dr Kadarla Rohith Kumar
Asst Professor, Department of Pharmacy Practice, Sree Chaitanya institute of Pharmaceutical Sciences, Thimmapur, Karimnagar, Telangana, India-505527