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 DDMS is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with positive radiological findings. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses.
Here, we report a case of a year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis. Magnetic resonance imaging of the brain showed unilateral atrophy of the left cerebral hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence.
These findings were suggestive of the diagnosis of DDMS. 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.
It has been reported that DDMS is caused by cerebral insult that may occur in utero when the maturation of calvarium has not been completed, or during early life due to brain damage usually traumatic. The clinical findings may be of variable degree according to the extent of the brain injury.
There was no history of head trauma. The convulsion was followed by decreased movement of the right side of the body and drooling of saliva from the right side occasionally, for which the patient was given some native medication.
Birth history was uneventful. However, her sibling had a history of generalized tonic-clonic convulsions, for which she was taking some antiepileptic drug for 2 years. The patient did not attend school. On examination, the child had very poor cognitive function and was undernourished. Positive findings were — microcephaly — severe mental retardation massson an intelligence quotient of 40, right-sided facial palsy, and sandal gap in both the lower limbs.
Sandal gap has not been a positive finding in any of the other reported cases of DDMS. Examination of the central nervous system revealed hemiparesis on the right side. Examination of other systems was unremarkable.
Complete blood count revealed moderate anemia.
Renal and liver function tests were normal. An X-ray of paranasal sinuses was done which was normal. There was subtle thickening of the left hemicranium noted, measuring 4. There was midline shift of 4 mm ipsilaterally. There was also mild atrophy of the left cerebral peduncle. 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 and prominence of sulci over the left cerebral hemisphere. There is also compensatory thickening of the skull vault. 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.
Birth trauma, hypoxia, intracranial hemorrhage, tumors, infections, and prolonged febrile seizures after birth are important peri- and post-natal causes. It can also be due to decreased carotid artery blood flow due to coarctation of aorta.
There are two types of DDMS — congenital infantile and acquired.
This feature differentiates it from cerebral hemiatrophy which occurs in early life. The atrophied cerebral hemisphere will have prominent sulcal spaces if the insult occurs after birth or after the end of sulcation. The clinical findings in DDMS may be of variable degree according to the extent of the brain injury: Seizures, davidofr asymmetry, contralateral hemiplegia or hemiparesis, mental retardation, and rarely, patients can also present with sensory symptoms and psychiatric disorders such as schizophrenia.
In the patient mentioned above, single episode of seizure was trailed by hemiparesis and mental deterioration. 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.
The compensatory cranial changes occur to take up the relative vacuum created by the atrophied or hypoplastic cerebral hemisphere. 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.
Important differential diagnosis includes — Sturge—Weber Syndrome, basal cell germinoma, Fishman syndrome, Silver—Russell syndrome, linear nevus syndrome, and Rasmussen encephalitis. Rasmussen encephalitis does not show calvarial changes, xavidoff Sturge—Weber syndrome additionally shows enhancing pial angiomas and cortical calcifications.
The treatment is symptomatic, and should target convulsion, hemiplegia, hemiparesis, and learning difficulties. Prognosis is better if hemiparesis occurs after the age of 2 years and without prolonged or repetitive seizures. National Center for Biotechnology InformationU. Journal List J Pediatr Neurosci v. Thakkar and Reema Haresh Dave. Author information Copyright and License information Disclaimer.
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, hemiparesis.
Introduction Dyke—Davidoff—Masson syndrome DDMS is described as skull radiographic and pneumatoencephalographic daavidoff 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. Open in a separate window. Discussion It has been reported that DDMS is caused by cerebral insult that may occur in utero when the maturation of calvarium has not been completed, or during early life due to brain damage usually traumatic.
Conflicts of interest There are no conflicts of interest. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Yerdelen D, Zafer F. Dyke—Davidoff—Masson syndrome in children. Internet J Pediatr Neonatol. Indian J Radiol Imaging. A cerebral cause of arthrogryposis: Disorders of cortical formation: Support Center Support Center. Please review our mzsson policy.