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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Birth trauma, hypoxia, intracranial hemorrhage, tumors, infections, and prolonged febrile seizures after birth are important peri- and post-natal causes. Ann Acad Med Singapore. The atrophied cerebral hemisphere will have prominent sulcal spaces if the insult occurs after birth or after the end of dqvidoff.

Magnetic resonance imaging of the brain showing diffuse atrophy of the left cerebral hemisphere with dilatation of the left lateral ventricle with compensatory thickening of the skull vault.

Prognosis is better if cyke occurs after the age of 2 years and without prolonged or repetitive seizures. Case 4 Case 4.

More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning disability, and speech and language disorders. Indian J Radiol Imaging. Degenerative diseases and hydrocephalus. It can also be due to decreased carotid artery blood flow due to coarctation of aorta. There was midline shift of 4 mm davidff. The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere.

The patient may present with seizure and cerebral imaging may show calcified cortex and hemiatrophy. Edit article Share article View revision history. Footnotes Source of Support: CT maszon the brain revealed characteristic features diagnostic of infantile type of cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome. Case 3 Case 3.


Sign in to access your subscriptions Sign in to your personal account. Sign in to save your search Sign in to your personal account. The clinical findings may be of variable degree according to the extent of the brain injury.

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Dyke-Davidoff-Masson Syndrome. | Congenital Defects | JAMA Neurology | JAMA Network

Copyright American Medical Association. Cerebral hemiatrophy, Dyke—Davidoff—Masson syndrome, hemiparesis. Please review our privacy policy. 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.

Seizures can be focal or generalized.

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

Sign in to make a comment Sign in dvidoff your personal account. Purchase access Subscribe now. He responded well to the drug followed by sessions of physiotherapy. Our website uses cookies to enhance your experience.

Abstract 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. Discussion It has been reported that DDMS is caused by cerebral insult that may occur in utero when the maturation of calvarium has not been completed, or during early life due to brain damage usually traumatic.

Dyke-Davidoff-Masson Syndrome

Purchase access Subscribe to JN Learning for one year. In our case, the findings of left cerebral davudoff with enlarged cortical sulci, microcephaly, and presentation at the age of 14 months reflect an onset of brain insult after the completion of sulci formation, probably of vascular origin involving left middle cerebral artery. Disorders of cortical formation: Cases and figures Imaging differential diagnosis. Natural history of acute hemiplegia of childhood.

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Thakkar and Reema Haresh Dave. Open in a separate window. Dyke—Davidoff—Masson syndrome DDMS is described as skull radiographic and pneumatoencephalographic changes in their series of nine patients whose clinical characteristics included hemiparesis, seizures, facial asymmetry, and mental retardation which was proposed in by Dyke et al. Case 6 Case 6. Articles Cases Courses Quiz.

Prognosis is better if the onset of hemiparesis is after 2 years of age and in absence of prolonged or recurrent seizure. National Center for Biotechnology InformationU. A plain and contrast CT of the brain was done which dyme atrophy of left cerebral hemisphere with dilatation of ddavidoff ipsilateral ventricle, widening of sulci and sylvian fissure on the same side.

Along with drugs, physiotherapy, occupational therapy, and speech therapy play a significant role in long-term management of the child. A month-old male child, born full term, to non-consanguineous parents, presented with recurrent generalized seizures for last 4 months. A rare neurocutaneous syndrome. The treatment is symptomatic, and should target convulsion, hemiplegia, hemiparesis, and learning difficulties.