Ntenga Syndrome: About an Observation

Abstract

Ntenga syndrome, is one of the highly epileptogenic, non-metabolic craniopathy whose aetiology is not yet known. This syndrome makes a differential diagnosis with that of Morgagni-Stewart-Morel which is rare and / or rarely mentioned in current clinical practice (entity made of frontal hyperostosis, neuropsychiatric and endocrine disorders). We report here a 58 years old female patient from Lubumbashi/ Democratic republic of Congo, followed for several years for multiform seizures, in whom the explorations of a status epilepticus, made possible to set up a new syndromic entity, called Ntenga syndrome made of a symptomatic triad (persistent multiform epileptic seizures, absence of endocrine disorder, hyperostosis frontalis interna). To date, a therapeutic protocol made of valproic acid and levetiracetam has significantly reduced to one seizure per month or even every 2 months. I think it is not without interest to report a very rare and / or new entity in the clinic.