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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There is also compensatory thickening of the skull vault. Cerebral hemiatrophy and homolateral hypertrophy dykr the skull and sinuses. He responded well to the drug followed by sessions of physiotherapy.
Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. The bilateral carotid pulsations were normal with no bruit. Important differential diagnosis includes — Sturge—Weber Syndrome, basal cell germinoma, Fishman syndrome, Silver—Russell syndrome, linear nevus syndrome, and Rasmussen encephalitis.
Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children
Support Center Support Center. A month-old male davidff, born full term, to non-consanguineous parents, presented with recurrent generalized seizures for last 4 months. There are two types of DDMS — congenital infantile and acquired.
Davidiff case studies and deductions from dermatoglyphics. He had delayed milestones of development in the form of not able to stand or walk and not spoken a word. The distinguishing features are the presence of port-wine facial nevus, intracranial tramtrack calcification, and the absence of midline shift.
The clinical findings may be of variable degree according to the extent of the brain injury. The surface of the hemisphere remains smooth and uninterrupted until early in the fourth month of gestation. Internet J Pediatr Neonatol. J Neurosci Rural Pract. Articles Cases Courses Quiz. Schweizer Archiv fur Neurologie und Psychiatrie.
Unable to process the form. Case Report A month-old male child, born full term, to non-consanguineous parents, presented with recurrent generalized seizures for last 4 months.
Nil Conflict of Interest: 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 means of suitable imaging CT and the treatment should focus on optimum control of seizures, revision of drug doses from time to time, and domiciliary physiotherapy. Prognosis is better if hemiparesis occurs after the age of 2 years and without prolonged or repetitive seizures.
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. Sturge-Weber syndrome Sturge-Weber syndrome. The human brain davidotf 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.
Sandal gap has not been a positive finding in any of the other reported cases of DDMS. Edit article Share article View davidfof history. Dyke—Davidoff—Masson syndrome DDMS is described mzsson 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 dtke al.
Dyke-Davidoff-Masson syndrome DDMS refers to atrophy or hypoplasia of one cerebral hemisphere hemiatrophy davidofr, which is usually due to an insult to the developing brain in fetal or early childhood period. About Blog Go ad-free. We present a case of Dyke Davidoff-Masson-syndrome with typical clinical and imaging features. Case 1 Case 1. Vision and hearing were normal, and cranial nerves were intact.
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.
Read it at Google Books – Find it at Amazon. 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.
More commonly they present with recurrent seizures, facial asymmetry, contralateral hemiplegia, mental retardation or learning disability, and speech and language disorders. A rare neurocutaneous syndrome. 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. Clinical presentations include variable degree of facial asymmetry, seizures, contralateral hemiparesis, mental retardation, learning disabilities, impaired speech, etc.
The treatment is symptomatic, and should target convulsion, hemiplegia, hemiparesis, and learning difficulties. Patients with DDMS usually present with refractory seizures dyyke the treatment should focus on control of the seizures with suitable anticonvulsants. Prognosis is better if the onset of hemiparesis is after 2 years of age and in absence of prolonged or recurrent seizure.
The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Cases and figures Imaging differential diagnosis. Open in a separate window.