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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Yerdelen D, Zafer F. Sign in to customize your interests Sign in to your personal account. Seizures can be focal or generalized.

Cerebral hemiatrophy can be of two types, infantile congenital and acquired. Footnotes Source of Support: Here, we report a case of a year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis.

These masosn and radiological findings are suggestive of DDMS. Unable to process the form.

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

It can also be due to decreased carotid artery blood flow due to coarctation of aorta. Purchase access Subscribe now. Coarctation of midaortic arch presenting with monoparesis.

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.


Rasmussen encephalitis does not show calvarial changes, and Sturge—Weber syndrome additionally shows enhancing pial angiomas and cortical calcifications. Journal List J Pediatr Neurosci v. Basal ganglia germinoma with cerebral hemiatrophy. Dykd a free personal account to make a comment, download free article PDFs, sign up for alerts and more. Development of mason Nervous system. Koshy B, Surendrababu NR. Please review our privacy policy. This condition is to be differentiated from Basal ganglia germinoma, Sturge Weber syndrome, Silver- Russel syndrome, Linear nevus syndrome, Fishman syndrome, and Rasmussen encephalitis.

Cerebral hemiatrophy and homolateral hypertrophy of the skull maason sinuses. Edit article Share article View revision history.

Age of presentation depends on time of insult and characteristic changes may be seen only in adolescence or adult.

Thakkar and Reema Haresh Dave. The surface of the hemisphere remains smooth and uninterrupted until early davidogf the fourth month of gestation. Internet J Pediatr Neonatol. Open in a separate window. Sign in to save your search Sign in to your personal account. A month-old male child presented with recurrent generalized seizures, spastic hemiplegia, microcephaly and had developmental delay in motor and speech domains.

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.


Read it at Google Books – Find it at Amazon. There was subtle thickening of the left hemicranium noted, measuring 4. There is also compensatory thickening of the skull vault. Case 6 Case 6. The patients have seizure disorder, mental retardation, and hemiparesis.

Clinical presentation Radiographic features History and etymology Differential diagnosis References Images: Computed tomography in cerebral hemiatrophy.

Dyke-Davidoff-Masson syndrome

Birth history was uneventful. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. It is characterised by: The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere. Davidocf human brain reaches half of its adult size during the first year of life and three fourth of the adult size is attained by the end of 3 years.

National Center for Biotechnology InformationU. Dyke-Davidoff-Masson Syndrome manifested by seizure in late childhood: The bilateral carotid pulsations were normal with no bruit. However, her sibling had a history of generalized tonic-clonic convulsions, for which she was taking some antiepileptic drug for 2 years.