Indian Pediatrics 2008; 45:927-928
Nigam Prakash Narain
From the Department of Pediatrics, Patna Medical College, Patna, Bihar, India.
Correspondence to: Dr. Nigam Prakash Narain, Associate
Professor, Department of Pediatrics, Patna Medical College,
Manuscript received: April 30, 2007; Initial review
completed: June 13, 2007;
First description of Dyke-Davidoff-Masson syndrome (DDMS) dates back to 1933, when Dyke, Davidoff and Masson described the plain skull radiographic and pneumatoencephalographic changes in a series of nine patients(1). Since then only few pediatric cases of DDMS have been reported.
An 18-month-old female baby, born full term, presented with recurrent tonic seizures of right half of body since the age of 6 month. There was no history of significant antenatal or perinatal complication. Neurological examination revealed right-sided spastic hemiparesis, brisk deep tendon reflexes, and extensor plantar response. The baby could not stand without support and spoke bisyllables only. There was no neurocutaneous marker or asymmetry of face or body and head circumference was normal. Vision and hearing were normal and cranial nerves were intact. Hematological profile and cerebrospinal fluid examination were normal. Computed Tomography (CT) scan of head revealed hemiatrophy of left cerebral hemisphere, dilatation of the left lateral ventricle, widening of ipsilateral sulci, a well defined cystic lesion and calvarial expansion on the left side. There was shift of the midline to the left. We made a diagnosis of Dyke-Davidoff-Masson syndrome.
DDMS is a rare condition characterized clinically by variable degrees of facial asymmetry, seizures, contralateral hemiparesis, mental retardation and learning disabilities in association with the classical radiological findings of asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral osseous hypertrophy and hyperpneumati-zation of sinuses(1-4).
Both sexes and any of the hemispheres may be affected but male gender and left hemisphere involvement are more frequent. Age of presentation depends on time of neurologic insult and characteristic changes may be seen only in adolescence. The clinical findings may be of variable degree depending on the extent of the brain injury. Varying degrees of atrophy of one half of body, sensory loss, speech and language disorder, mental retardation or learning disability and psychiatric manifestations like schizophrenia may also be present. In the present case, the findings of dilated cortical sulci and widening of ipsilateral diploi reflect a late onset of brain insult probably of vascular origin involving left middle cerebral artery.
A proper history, thorough clinical examination and radiologic findings provide the correct diagnosis. The condition needs to be differentiated from Basal ganglia germinoma, Sturge Weber syndrome, Linear nevus syndrome, Fishman syndrome, Silver-Russell syndrome and Rasmussen encephalitis(5,6).
A possible etiological relation between cerebral atrophy and seizures has been reported in two different studies from India(7,8). Prognosis is better if hemiparesis occurs after the age of 2 yrs and in absence of prolonged or recurrent seizures. Children with intractable disabling seizures and hemiplegia are the potential candidates for hemispherectomy with a success rate of 85% in carefully selected cases.
Contributors: NPN diagnosed this case, supervised the management and drafted the manuscript; he will act as guarantor of the paper. BN and RK were involved in patient management and review of literature.
Competing interests: None stated.