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

Case 4 Case 4. Ind J Radiol Imag. Temporo-spatial analysis define epileptogenic and functional zone in a case of DDMS. Get free access to newly published articles. More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning disability, and speech and language disorders. In our case, the findings of left cerebral hemiatrophy 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.

Author information Copyright and License information Disclaimer. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. Support Center Support Center.

Dyke-Davidoff-Masson syndrome | Radiology Reference Article |

Footnotes Source of Support: Introduction 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. Open in a separate window. 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.

Cerebral hemiatrophy, Dyke—Davidoff—Masson syndrome, massin. 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.

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This dvaidoff differentiates it from cerebral hemiatrophy which occurs in early life. Sign in to access your subscriptions Sign in to your personal account. Five case studies and deductions from dermatoglyphics. Examination of the central nervous system revealed hemiparesis on the right side. The main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, massom prolonged febrile seizure. These clinical and radiological findings are suggestive of DDMS.

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. Dyke—Davidoff—Masson syndrome DDMS is an uncommon condition, in which the diagnosis is mainly done by various davivoff presentations along with positive radiological findings.

A month-old male child presented with recurrent generalized davdoff, spastic hemiplegia, microcephaly and had developmental delay in motor and speech domains. In some sources, it is equated to hemispheric infarctionwhereas in other sources any cause of cerebral hemiatrophy is included.

Please review our privacy policy. There is also thickening of the calvarium on the left side. CT of Cerebral hemiatrophy. CT of the brain revealed characteristic features diagnostic of infantile type of cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome.

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

Create a personal account to register for email alerts with links to free full-text articles. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Disorders of cortical formation: Age of presentation depends on time of insult and characteristic changes may be seen only in adolescence or adult. Privacy Policy Terms of Use. Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.


Journal List J Pediatr Neurosci v. Djke syndrome in children.

National Center for Biotechnology InformationU. Edit davidofg Share davidofr View revision history. A proper history, thorough clinical examination, and radiologic findings provide the correct diagnosis. National Center for Biotechnology InformationU.

He was seizure free for next 3 months but then unfortunately lost for follow-up. 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.

This syndrome is usually seen during adolescence; however, it can also be seen in childhood. Get free access to newly published articles Create a personal account or sign in to: The surface of the hemisphere remains smooth and uninterrupted until early in the fourth month of gestation. The imaging features include unilateral hemispheric atrophy without any calvarial changes. Birth dke was uneventful. Sign in to save your search Sign in to your personal account.

Dyke-Davidoff-Masson syndrome

It can also be due to decreased carotid artery blood flow due to coarctation of aorta. There was also shift of midline to left and thickening of calvarium on the left side [ Figure 1 ]. Purchase access Subscribe to the journal.

Seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, mental retardation, and rarely, patients can also present with sensory symptoms and psychiatric disorders such as schizophrenia. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.