Dyke-Davidoff-Masson syndrome is characterized by unilateral atrophy of cerebral hemispheres, enlargement of the ipsilateral sulci, ventricles, and cisternal sp. Dyke-Davidoff-Masson syndrome is a rare condition of unknown frequency resulting from brain injury due to a multitude of causes; especially in. Dyke-Davidoff-Masson Syndrome (DDMS) refers to atrophy or hypoplasia of one cerebral hemisphere. (hemiatrophy) which is secondary to brain insult.

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Nil Conflict of Interest: A cerebral cause of arthrogryposis: CT of the brain revealed characteristic features diagnostic of infantile type of cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome. On examination, the child had very poor cognitive function and was undernourished.

Case Report A month-old male child, born full term, to non-consanguineous parents, presented with recurrent generalized seizures for last 4 months. Temporo-spatial analysis define epileptogenic and functional zone in a case of DDMS.

Davidovf of the Nervous system. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. This condition is to be differentiated from Basal ganglia germinoma, Sturge Weber syndrome, Silver- Russel syndrome, Linear nevus syndrome, Fishman syndrome, and Rasmussen encephalitis.

Purchase access Subscribe to JN Learning for one year. Abstract Dyke—Davidoff—Masson syndrome DDMS is an uncommon condition, in which the diagnosis is mainly done by various dhke presentations along with positive radiological findings. Sturge-Weber syndrome Sturge-Weber syndrome.

Sandal gap has not been a positive finding in any of the other reported cases of DDMS. Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. Along with drugs, physiotherapy, occupational therapy, and speech therapy play a significant role in long-term management of the child.


Sensory loss and psychiatric manifestations like schizophrenia had been reported rarely.

Support Radiopaedia and see fewer ads. Clinical presentations include variable degree of facial asymmetry, seizures, contralateral hemiparesis, mental retardation, learning disabilities, impaired speech, etc.

Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children

Discussion InDyke, Davidoff and Masson first described the syndrome in plain radiographic and pneumoencephalographic changes in a series of nine patients.

Examination of the central nervous system revealed hemiparesis on the right side.

National Center for Biotechnology InformationU. The imaging features include unilateral hemispheric atrophy without any calvarial changes. Here, we report a case of a year-old female child who msason with a single episode of convulsion, mental retardation, and contralateral hemiparesis. Magnetic resonance imaging of the brain showed unilateral atrophy of the left cerebral hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence.

Dyke-Davidoff-Masson syndrome DDMS refers to atrophy or hypoplasia of one cerebral hemisphere hemiatrophywhich is usually due to an insult to the developing brain in fetal or early childhood period. It usually presents with intractable focal epilepsy and cognitive defects in children. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.

Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children

Prognosis is better if the onset of hemiparesis is after 2 years of age and in absence of prolonged or recurrent seizure. The patient may present with seizure and cerebral imaging may show calcified cortex and hemiatrophy. The main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizure. A plain and contrast CT of the brain was done which revealed atrophy of left cerebral hemisphere with dilatation of the ipsilateral ventricle, widening of sulci and sylvian fissure on the same side.


Internet J Pediatr Neonatol. Renal and liver function tests were normal. These findings were suggestive of the diagnosis of DDMS. Case 1 Case 1. Case 5 Case 5.

The human brain reaches half of its adult size during the first year of life and three fourth of the adult size is attained by the end of 3 years. Ind J Radiol Imag.

Dyke-Davidoff-Masson syndrome

The clinical findings may be of variable degree according to the extent of the brain injury. Read it at Google Books – Massoon it at Amazon.

There was also mild atrophy of the left cerebral peduncle. The patients have seizure disorder, mental retardation, and hemiparesis. In some sources, it is equated to hemispheric infarctionwhereas in other sources any cause of cerebral hemiatrophy is included. We present a case of Dyke Davidoff-Masson-syndrome with typical clinical and imaging features.

Dyke—Davidoff—Masson syndrome DDMS is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with positive radiological findings. In the patient mentioned above, single episode of seizure was trailed by hemiparesis and mental deterioration.