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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Eavidoff 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 mason 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.

In the patient mentioned above, single episode of seizure was trailed by hemiparesis and mental deterioration.

Dyke-Davidoff-Masson syndrome

Birth trauma, hypoxia, intracranial hemorrhage, tumors, infections, and prolonged febrile seizures after birth are important peri- and dykf causes. Clinical presentation Radiographic features History and etymology Differential diagnosis References Images: Positive findings were — microcephaly — severe mental retardation with an intelligence quotient of 40, right-sided facial palsy, and sandal gap in both the lower limbs.


Clinical presentations include variable degree of facial asymmetry, seizures, contralateral hemiparesis, mental retardation, learning disabilities, impaired speech, etc. Synonyms or Alternate Spellings: 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.

Nil Conflict of Interest: Sometimes multiple anticonvulsants are in use.

Dyke-Davidoff-Masson syndrome

More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning mason, and speech and language disorders.

Here, we report a case of a year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis. It can also be due to decreased carotid artery blood flow due to coarctation of aorta.

Graham A, Molnar Z. Footnotes Source of Support: Discussion InDyke, Davidoff and Masson first described the syndrome in plain radiographic and pneumoencephalographic changes in a series of nine patients. Dyke—Davidoff—Masson syndrome in children. The imaging features include unilateral hemispheric atrophy without any calvarial changes. There was also shift of midline to left and thickening of calvarium on the left side [ Figure 1 ].

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Case 5 Case 5. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Sturge-Weber syndrome Sturge-Weber syndrome. Purchase access Subscribe to JN Learning for one year.

Imaging spectrum includes varying degrees of cerebral hemiatrophy of the affected hemishpere with dilatation of the ipsilateral lateral ventricle and ipsilateral sulcal prominence accompanied by homolateral hypertrophy of the skull and sinuses. A proper history, thorough clinical examination, and radiologic findings provide the correct diagnosis. CT of Daidoff hemiatrophy.


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

Get free access to newly published articles. Thakkar and Reema Haresh Dave. There was daviddoff history of head trauma. A cerebral cause of arthrogryposis: The atrophied cerebral hemisphere will have prominent sulcal spaces if the insult occurs after birth or after the end of sulcation.

Sign in to save your search Sign in to your personal account. MR of craniocerebral hemiatrophy. Sign in to customize your interests Sign in to your personal account. As hemispherectomy is not available even in many urban tertiary care centers, it is very important for a rural neurologist or pediatrician to diagnose the condition early by xyke of suitable imaging CT and the treatment should focus on optimum control of seizures, revision of mwsson doses from time to time, and domiciliary physiotherapy.