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.

Keywords: Calvarial thickening; Cerebral hemiatrophy; Dyke-Davidoff-Masson syndrome.

Fig. 1

T2-weighted axial MRI showing cerebral hemiatrophy on the right side of the brain at the level of the basal ganglia (a) and at the supraganglionic level (b).

Fig. 2

MRI of the brain showing frontal sinus hypertrophy in an axial T2-weighted sequence (thick white arrow; a) along with calvarial thickening in an axial T1-weighted sequence (thick black arrow; b). Crossed cerebellar hemiatrophy is evident on the left side in axial T2-weighted (thin white arrow; c) and coronal T2-weighted images (thin black arrow; d).