Herniati H Alimadong,Hadia Angraini,Urfianty ,Eka Yusuf Inra Kartika
Depertemen Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Hasanuddin Departemen Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Hasanuddin
Background : Dyke-Davidoff-Masson syndrome (DDMS) or Cerebral hemiatrophy is condition characterized by seizures, contralateral hemiplegia or hemiparesis, facial asymmetry and mental retardation or learning disability. The features may be present in varying combinations and degrees of severity. The findings are due to cerebral injury that may occur in utero or early in life.
Case Presentation Summary : A 7-year and 10-months old female child presented to Hasanuddin University Hospital with recurrent generalized tonic-clonic seizures since two months prior to admission. There was history of febrile convulsion at 5- months, no history of significant antenatal or perinatal complication. Physical examination revealed right-sided spastic, hemiparesis with brisk deep tendon reflexes, other systemic examination being normal. There was no neurocutaneus marker or asymmetry of the face or body. Hematological profile examination were normal. The EEG revealed focal seizure. CT Scan of the head revealed hemiatrophy of left cerebral hemisphere, dilatation of left lateral ventricle and ipsilateral sulcal prominence suggestive of DDMS. The patient were treated by using antiepileptic drugs and physiotherapy.
Learning Points/Discussion : - Patients with DDMS usually present with refractory seizures and the treatment should focus on control of the seizures with suitable anticonvulsant. - Prognosis is better if the onset of hemiparesis is a after 2 years af age and in absence of prolonged or recurrent seizure.