Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature

Abstract

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition usually diagnosed in paediatric patients with clinical features of hemiparesis, seizures, mental retardation and contralateral cerebral hemiatrophy on neuroimaging. This report follows the case of a 22-year-old man presenting with seizures and hemiatrophy and hemiparesis. On review of cases the most common neuroimaging findings were cerebral hemiatrophy (100%) followed by hemicalvarial thickening (71.4%) and hyperpneumatisation of sinuses (71.4%). Apart from our patient, all nine cases with data on epilepsy control had drug-resistant epilepsy. The onset of seizures in adulthood, block vertebra, short stature, absence of mental retardation and well-controlled epilepsy on monotherapy makes our case exceptional-even bringing to mind the possibility of a DDMS variant. This report exhaustively reviews the wide range of clinical and radiological manifestations of DDMS in the adult, thereby adding to the literature on an unusual syndrome that causes significant neurological morbidity.

Keywords: epilepsy and seizures; neurology.

Figure 1

(A–C) Different views showing hemiatrophy of the patient’s left side.

Figure 2

(A–B) Axial T1-weighted MRI showing hemicalvarial thickening (white arrow) and hemiatrophy of right cerebral hemisphere. (C–E) Axial T2-weighted MRI showing volume loss on the right side of the brainstem. (F) Block vertebra (white arrowhead), T7–T8 vertebrae, with residual intervertebral disc material and exuberant spur formation abutting the thecal sac.