Adult Presentation of Dyke-Davidoff-Masson Syndrome: A Case Report

Abstract

Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease which is clinically characterized by hemiparesis, seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. This disease is a rare entity, and it mainly presents in childhood. Adult presentation of DDMS is unusual and has been rarely reported in the medical literature. Key Messages: DDMS is a rare disease of childhood. However, it should be kept in mind as a diagnostic possibility in an adult who presents with a long duration of progressive hemiparesis with seizures and mental retardation. Cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses are diagnostic for this illness on brain imaging.

Dyke–Davidoff–Masson syndrome in a Nigerian

“…In Rasmussen's syndrome, the calvarial changes are not found. Basal ganglia germinoma presents with similar clinical features, but brain imaging reveals cystic areas, focal hemorrhages, and mild surrounding edema along with calvarial changes [7]. In Sturge–Weber syndrome, brain imaging shows enhancing pial angiomas and cortical calcifications in addition to the clinical features of the phacomatosis.…”

“…In Sturge–Weber syndrome, brain imaging shows enhancing pial angiomas and cortical calcifications in addition to the clinical features of the phacomatosis. Silver–Russell syndrome is characterized by the classical facial phenotype (triangular face; small, pointed chin; broad forehead; and thin, wide mouth), poor growth with delayed bone age, clinodactyly, hemihypertrophy with normal head circumference, and normal intelligence [7]. Fishman syndrome or encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome including unilateral cranial lipoma with lipodermoid of the eye, which usually presents with seizures.…”

“…Fishman syndrome or encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome including unilateral cranial lipoma with lipodermoid of the eye, which usually presents with seizures. Neuroimaging, however, shows a calcified cortex and hemiatrophy [7]. Linear nevus sebaceous syndrome (a.k.a.…”

“…If seizures are drug-resistant, they can be treated surgically with hemispherectomy, which has an 85% chance of rendering the patient seizure free [7]. Long-term management also includes physiotherapy, occupational therapy, and speech therapy.…”

The Clinico-Radiological Spectrum of Dyke-Davidoff-Masson Syndrome in adults

“…Generally the disease is diagnosed in childhood. There are single case reports in the literature for diagnosis in adulthood [5,6].…”