Severe Neurological Involvement In Tuberous Sclerosis: A Report Of Two Cases And A Review Of The African Literature

Abstract

Tuberous sclerosis (TS) is a genetic disorder characterised by the triad of cutaneous lesions, epilepsy and mental retardation. TS is known to have a wide clinical spectrum, with some affected individuals having only the cutaneous manifestations, normal IQ and no seizures, while others are severely affected having intractable seizures and profound mental retardation. A report of two Nigerian children with TS managed at the University College Hospital, Ibadan, Nigeria is presented. Both had severe neurological manifestations of the disease and a sub clinical affectation was found in a first degree relative in one of them.



African Journal of Neurological Sciences Vol. 26 (2) 2007: pp. 102-108