Dyke–Davidoff–Masson Syndrome in Adulthood

Abstract

Dyke–Davidoff–Masson syndrome is a rare neurological condition characterized by unilateral cerebral atrophy with compensatory thickening of the cranial vault, usually resulting from early brain injury. The diagnosis is typically established in childhood, with its identification in adulthood being uncommon. We describe the case of a 49-year-old male patient with a two-year history of recurrent headaches in the right temporoparietal region. Neurological examination revealed mild right hemiparesis and hemihypoesthesia, associated with ipsilateral conjunctival hyperemia and lacrimation. Based on these findings, the hypothesis of short-lasting unilateral neuralgiform headache attacks was initially considered. Neuroimaging revealed left cortico-subcortical encephalomalacia, ipsilateral cerebral hemiatrophy, and compensatory thickening of the cranial vault, findings compatible with Dyke–Davidoff–Masson syndrome. This case highlights the importance of neuroimaging in patients with atypical headaches and subtle focal neurological deficits.