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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Development of the Nervous system. Clinical presentation Radiographic features History and etymology Differential diagnosis References Images: We present a case of Dyke Davidoff-Masson-syndrome with typical clinical and imaging features.

Create a free personal account to access your subscriptions, sign up for alerts, and more. Yerdelen D, Zafer F. J Neurosci Rural Pract. Seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, mental retardation, and rarely, patients can also present with sensory symptoms and psychiatric disorders such as schizophrenia. Read it at Google Books – Find it at Amazon.

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

The patients have seizure disorder, mental retardation, and 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. He was seizure free for next 3 massoh but then unfortunately lost for follow-up.


Cerebral hemiatrophy can be of two types, infantile congenital and acquired. Complex partial seizure with secondary generalization also had been reported. The convulsion was followed by decreased movement of the right side of the body and drooling of saliva from the right side occasionally, for which the patient was given some native medication.

Prognosis is better if the onset of hemiparesis is after 2 years of age and in absence of prolonged or recurrent seizure. These clinical and radiological findings are suggestive of DDMS.

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. Dyke—Davidoff—Masson syndrome in children. Create a davidfof personal account to download free article PDFs, sign up for alerts, customize your interests, and more.

Thakkar and Reema Haresh Dave. Nil Conflict of Interest: Cases and figures Imaging differential diagnosis.

Magnetic resonance imaging of the brain showed unilateral atrophy of the left davidff hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence. Discussion InDyke, Davidoff and Masson first described the syndrome in plain radiographic and pneumoencephalographic changes in a series of nine patients. Graham A, Molnar Z. The distinguishing features are the presence of port-wine facial nevus, intracranial tramtrack calcification, and the absence of midline shift.

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

The imaging features include unilateral hemispheric atrophy without any calvarial changes. There was midline shift of 4 mm ipsilaterally. Renal and liver function tests were normal. Magnetic resonance imaging of the brain showing diffuse atrophy of the left cerebral hemisphere with dilatation of the left lateral ventricle and prominence of sulci over the left cerebral hemisphere.


Schweizer Archiv fur Neurologie und Psychiatrie. Edit article Share article View revision history. Loading Stack – 0 images remaining. Get free access to newly published articles Create a personal account or sign in to: Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more.

Thank you for updating your details. There is also thickening of the calvarium on the left side. The surface of the hemisphere remains smooth and uninterrupted until early in the fourth month of gestation.

You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Degenerative diseases and hydrocephalus. A rare neurocutaneous syndrome. Basal ganglia germinoma with cerebral hemiatrophy.