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

Ind J Radiol Imag. Coarctation of midaortic arch presenting with monoparesis. Complete blood count revealed moderate anemia. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. Elevation of the petrous ridge and ipsilateral falcine displacement may also be present. It usually presents with intractable focal epilepsy and cognitive defects in children.

The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere. There are two types of DDMS — congenital infantile and acquired.

The imaging features include unilateral hemispheric atrophy without any calvarial changes. National Center for Biotechnology InformationU.

There was subtle thickening of the left hemicranium noted, measuring 4. 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. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.


This article has been cited by other articles in PMC. Internet J Pediatr Neonatol. The syndrome had been documented mainly in adolescents and adults. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Create a free personal account to maswon free article PDFs, sign up for alerts, and more.

Sign in to customize your interests Sign in to your personal account. Koshy B, Surendrababu NR.

Journal List J Pediatr Neurosci v. When DDMS develops early in life during the first 2 yearscertain cranial changes such as ipsilateral masaon 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. This feature differentiates it from cerebral hemiatrophy which occurs in early life.

Examination of other systems was unremarkable.

There was no history of significant antenatal or perinatal complications. Degenerative diseases and hydrocephalus.

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

The patients have seizure disorder, mental retardation, and hemiparesis. Ann Acad Med Singapore. A rare neurocutaneous syndrome. There is also thickening of the calvarium on the left side. 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.


Sign in to save your search Sign in to your personal account. Birth history was uneventful. Development of the Nervous system. In addition to CT xyke 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.

Check for errors and try again. Disorders of cortical formation: Case 4 Case 4. Cases and figures Imaging differential diagnosis.

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

Case 1 Case dyyke. Natural history of acute hemiplegia of childhood. Synonyms or Alternate Spellings: Dyke—Davidoff—Masson syndrome in children. In the patient mentioned above, single episode of seizure was trailed by hemiparesis and mental deterioration.

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