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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

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. Positive findings were — microcephaly — severe mental retardation with an intelligence quotient of 40, right-sided facial davicoff, and sandal gap in both the lower limbs. There is also compensatory thickening of the skull vault.

Complete blood count revealed moderate anemia. Case 6 Case 6. There are two types of DDMS — congenital infantile and acquired. Yerdelen D, Zafer F.

Dyke-Davidoff-Masson Syndrome

Temporo-spatial analysis define epileptogenic and functional zone in a case of DDMS. More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning disability, and speech and language disorders. When the brain fails to grow properly, the other structures grow inward resulting in increased width of diploic spaces, enlarged sinuses, and elevated orbital roof.


Elevation of the petrous ridge and ipsilateral falcine displacement may also be present. Introduction 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. Rasmussen encephalitis does not show calvarial changes, and Sturge—Weber syndrome additionally shows enhancing pial angiomas and cortical calcifications.


Copyright American Medical Association. Prognosis is better if hemiparesis occurs after the age of 2 years and without prolonged or repetitive seizures.

The main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizure. A month-old male child presented with recurrent generalized seizures, spastic hemiplegia, microcephaly and had developmental delay in motor and speech domains. Along with drugs, physiotherapy, occupational therapy, and speech therapy play a significant role in long-term management of the child.

Conflicts of interest There are no conflicts of interest.

An X-ray of paranasal sinuses was done which was normal. 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.

Support Center Support Center. Important differential diagnosis includes — Sturge—Weber Syndrome, basal cell germinoma, Fishman syndrome, Silver—Russell syndrome, linear nevus syndrome, and Rasmussen encephalitis.

Birth trauma, hypoxia, intracranial hemorrhage, tumors, infections, and prolonged febrile seizures after birth are important peri- and post-natal causes. CT of Cerebral hemiatrophy.

Loading Stack – 0 images remaining. There was no history of significant antenatal or perinatal complications. Sign in to access your subscriptions Sign in to your personal account.

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

Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. He was seizure free for next 3 months but then unfortunately lost for follow-up. On examination, the child had very poor cognitive function and was undernourished. 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.


Open in a separate window. 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 and ipsilateral falcine displacement. There was no history of head trauma.

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Dyke-Davidoff-Masson syndrome | Radiology Reference Article |

The bilateral carotid pulsations were normal with no bruit. Cases and figures Imaging differential diagnosis. There was subtle thickening of the left hemicranium noted, measuring 4. Create a free personal account to download free article PDFs, sign up for alerts, and more. There was also mild dyks of the left cerebral peduncle. CT of the brain revealed characteristic features diagnostic of infantile type of cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome.