The Duke–Davidoff–Masson syndrome (DDMS) is a rare neurological condition with unknown prevalence, globally. To date, <100 cases have been reported worldwide. We report the case of an 18‐year‐old patient admitted for status epilepticus seizure, and who presented a right hemiparesis, body asymmetry, joints ankylosis, and mental retardation. Brain CT‐scan revealed left hemisphere atrophy, skull bone thickening, and hyperpneumatization of the frontal sinuses; all consistent with DDMS. Seizures improved remarkably on Levetiracetam and Valproate. This is the first report of an unusual DDMS in Mali, and the diagnosis delay highlights the challenges for the management of these diseases in resource‐limited settings.
In resource-limited countries, epilepsy prevalence is underestimated and little is known about its risk factors.
Objectives: This study aimed to determine the prevalence and risk factors for epilepsy in six health districts (HD) in Mali.
Methods
A community-based cross-sectional and nested-case–control study was conducted in 180 villages with the highest number of suspicious epilepsy cases (SEC) in the six study HD. The SEC were observed as part of a Phase 1 screening conducted by community health workers (CHWs). For the nested case-control study, one case was matched with at least one control based on residence and age. A case of epilepsy was any person diagnosed with convulsive epilepsy after clinical assessment by a neurologist. A control was a person diagnosed as normal after neurological assessment by a neurologist. Data were collected on sociodemographic characteristics, familial and medical history of epilepsy, consanguinity, place of delivery, pre-term birth, length/type of delivery, and history of meningitis and cerebral malaria. A univariate and multivariate binomial logistic regression model was used to analyse factors associated with epilepsy.
Results
A total of 1,506 cases of epilepsy and 2,199 controls were enrolled in six HDs. The mean prevalence of epilepsy was 2‰, with the highest in Kenieba (3‰), a previously meso-endemic-onchocerciasis HD, and the lowest in Kadiolo (1.5‰), an hypo-endemic-onchocerciasis HD. Age [aOR=1.02 (95% CI 1.02-1.03)], history of cerebral malaria [aOR=11.41 (95% CI 8.86-14.85)], history of meningitis [aOR=1.95 (95% CI 1.16-3.29)], living in the HD of Tominian [aOR= 1.69 (95% CI 1.29-2.22)], delayed delivery [aOR= 3.21 (95% CI 2.07-5.07)] and dystocia [aOR= 3.37 (95% CI 2.03-5.73)] were all significantly associated with epilepsy.
Discussion/Conclusion
The prevalence of epilepsy (3‰) in a previously meso-endemic-onchocerciasis HD was much lower than the prevalence (13.35‰) documented in onchocerciasis endemic areas in 2000. This decrease epilepsy prevalence in the previously meso-endemic-region was induced by onchocerciasis and the reduction was due to an effective community direct treatment with ivermectin programme. Cerebral malaria and obstetrical complications were the main risk factors for epilepsy and interventions improving malaria prevention/treatment and optimising prenatal and obstetrical care need to be implemented to reduce incidence.
Duke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition
with unknown global prevalence. It typically manifests with body
asymmetry, drugs resistant epilepsy, mental retardation, cerebral
atrophy, skull bone thickening and hyperpneumatization of the frontal
sinuses. In this report, we present an unusual case of DDMS revealed by
status epilepticus.
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