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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Dyke-Davidoff-Masson Syndrome

Purchase access Subscribe to the journal. The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere.

In addition to CT findings described above, MRI demonstrates the gray-white matter loss with hyperintensities on T2-weighted images diffuse cortical and subcortical atrophy and asymmetry of the basal ganglia.

Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account. There was subtle thickening of the left hemicranium noted, measuring 4. Patients with DDMS dykw present with refractory seizures and the treatment should focus on control of the seizures with suitable anticonvulsants.

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. Cases and figures Imaging differential diagnosis. Case 3 Syke 3. Sturge-Weber syndrome Sturge-Weber syndrome. Read it daviodff Google Books – Find it at Amazon. Prognosis is better if hemiparesis occurs after the age of 2 years and without prolonged or repetitive seizures.

Koshy B, Surendrababu NR. 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.

There is also thickening of the calvarium on the left side. 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. Ind J Radiol Imag. Unable to process the form. Along with drugs, physiotherapy, occupational therapy, and speech therapy play a significant role in long-term management of the child.

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Sign in to access dyme subscriptions Sign in to your personal account. Complete blood count revealed moderate anemia.

Dyke-Davidoff-Masson syndrome | Radiology Reference Article |

Discussion InDyke, Davidoff and Masson first described the syndrome in plain radiographic and pneumoencephalographic changes in a series of nine patients. Neurological examination revealed right-sided spastic hemiparesis with brisk tendon reflexes and extensor planter response, other systemic examinations being normal.

The imaging features include unilateral hemispheric atrophy without any calvarial changes. The patients have seizure disorder, mental retardation, and hemiparesis. Author information Copyright and License information Disclaimer.

Age of presentation depends on time of insult and characteristic changes may be seen only in adolescence or adult. In some sources, it is equated to hemispheric infarctionwhereas in other sources any cause of cerebral hemiatrophy is included. When DDMS develops early in life during the first 2 yearscertain cranial changes such as ipsilateral hypertrophy of the skull and enlargement of sinuses occur, the elevations of the greater wing of sphenoid and the petrous ridge on the affected side davidlff ipsilateral falcine displacement.

The syndrome had been documented mainly in adolescents and adults. He was seizure free for next 3 months but then unfortunately lost for follow-up. Here, we report a case of a year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis. The patient did not attend school. Sign in vavidoff save your search Sign in to your personal account. National Center for Biotechnology InformationU.

Internet J Pediatr Neonatol.

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

Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses.

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cavidoff Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. This article has been cited by other articles in PMC. Journal List J Pediatr Neurosci v. A month-old male child, born full term, to non-consanguineous parents, presented with recurrent generalized seizures for last 4 months. InDyke, Davidoff and Masson first dabidoff the syndrome in plain radiographic and pneumoencephalographic changes in a series of nine patients.

Clinical presentations include variable degree of facial asymmetry, seizures, contralateral hemiparesis, mental retardation, learning disabilities, impaired dykr, etc. More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning disability, and speech and language disorders. Dyke—Davidoff—Masson syndrome DDMS is described as skull radiographic and pneumatoencephalographic changes in their series of nine patients whose clinical characteristics included hemiparesis, davjdoff, facial asymmetry, and mental retardation which was proposed in by Dyke et al.

Prognosis is better if the onset of hemiparesis is after 2 years of age and in absence of prolonged or recurrent seizure.

Computed tomography in cerebral hemiatrophy. Create a personal account to register for email alerts with links to free full-text articles. This syndrome is usually seen during adolescence; however, it can also be seen in childhood. On examination, the child had very poor cognitive function and was undernourished.

Case 4 Case 4. Complex partial seizure with secondary generalization also had been reported. Disorders of davidorf formation: Magnetic resonance imaging of the brain showed unilateral atrophy of the left cerebral hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence.