Sheer neurological form of tuberous sclerosis: about an observation at fann department of neurology in dakar (Senegal)

Ibrahima M Diallo, Ngor S Diagne, Soumaïla Boubacar, Marie E Ndong, Mohamed L Mansaré, Ousmane Cisse, Japhari Nyassinde, Moustapha Ndiaye, Amadou G Diop and Mouhamadou M Ndiaye

Tuberous sclerosis complex (TSC) is an important neurocutaneous syndrome to diagnose because of its phenotypic variability and the often serious nature of its manifestations. The pure neurological form of STB is rare in clinical practice. We report here a recent case of tuberous sclerosis with pure neurological symptomatology in a Senegalese child. This was a 2-year-old child with no prior personal history except for a delayed psychomotor development. No family history had been reported. He was received in neuropediatric consultation for partial motor seizures secondarily generalized for 15 months. The neurological and neuropsychological examination showed a mild cognitive deficit and a bilateral pyramidal syndrome. Despite complet clinical evaluation, there was no dermatological lesion. The rest of the clinical examination was strictly normal. The diagnosis of tuberous sclerosis has been made on the basis of neurological manifestations (refractory epileptic seizures), age of the patient, and characteristics of brain imagery and absence of other cause that can justify his illness. The patient was stabilized by carbamazepine and clonazepam. The evolution after 4 months was favorable with a rarefaction of the crises and the decrease of their intensities. Tuberous sclerosis must be evoked before any child with seizures refractory to the usual treatment. Early diagnosis helps to improve management and to consider genetic counseling within the family.

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