Introduction
Duke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition
with unknown global prevalence and incidence. This disease was first
described by three physicians, Cornelius G. Dyke, Leo M. Davidoff and
Clement M. Masson in 1933.1 DDMS is a
clinico-radiological recognized entity characterized by typical
manifestations including drugs resistant epilepsy, intellectual
disability, hemiparesis, skull bone thickening associated with cerebral
hemiatrophy and hyperpneumatization of paranasal and frontal sinuses on
neuroimaging.2 Some patients may present with
additional symptoms including cerebellar and basal ganglia atrophy, ear
malformations and neuropsychiatric disorders.3, 4DDMS’ etiologies are broadly categorized into congenital and
acquired.5 Congenital causes of DDMS include
intrauterine vascular injury and cerebral hemispheric hypoperfusion and
acquired causes mostly derive from birth trauma, periventricular
leukomalacia, cerebral hemorrhage, cerebral infarction, cerebral
infection, radiation, postictal cerebral hemiatrophy, and prolonged
febrile seizures.6, 7 Almost a century after the first
description, the pathogenic mechanism remains unclear and is subject to
controversy. To date, less than 100 cases have been reported worldwide
with only four cases in the African population.8-11 We
report here the case of an 18-year-old adolescent who was admitted in
our neurology clinic for convulsive status epilepticus and in which
clinical and laboratory findings were in favor of DDMS, the first case
in Mali.