Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Abstract

We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere. RESUME: Le role de I'hemispherectomie dans le prognostic de la maladie de Sturge-Weber-Dimitri Nous rapportons les cas de 12 patients atteints de la maladie de Sturge-Weber-Dimitri qui ont ete traites chirurgicalement entre Janvier 1975 et decembre 1987. Une hemispherectomie a ete effectuee chez 10 patients et 2 autres ont subi une lobectomie occipitale pour epilepsie refractaire au traitement. Toutes les interventions ont ete realisees entre Page de 3 et 20 mois, sauf chez deux patients ages de 8 et 9 ans respectivement. Le debut des crises convulsives se situait entre 2 et 8 mois chez tous les patients sauf chez deux qui avaient commence a 15 mois. II n'y a eu aucun deces en post-operatoire. On a du avoir recours a un shunt post-operatoire chez 3 des 12 patients (25%). On a obtenu un controle des crises pendant un an ou plus apres 1'intervention chez 11 des 12 patients. L'autre patient n'a pas eu de crise sous medication apres un suivi de moins de 12 mois. Nous n'avons pas observe de deterioration intellectuelle apres la chirurgie sauf chez 2 patients operes tardivement. Nous concluons que, chez les patients atteints du syndrome de Sturge-WeberDimitri qui presentent des crises convulsives refractaires au traitement dans les premiers six mois apres la naissance et dont Patteint hemispherique est unilateral, une resection precoce de Phemisphere atteint doit etre consideree.

Neuropsychology of childhood epilepsy: Pre‐ and postsurgical assessment

“…This was also true for age at seizure onset: overall, no correlation was found between age at onset of seizures (from 0 to 12 years) and postsurgical intellectual functioning as assessed mainly by the Wechsler scales. The same pattern was observed when correlations were computed between IQ and duration of seizures or age at surgery in a sample of 36 left‐hemispherectomized and 36 right‐hemispherectomized patients whose scores were reported in the articles [66‐68, 70, 73‐78, 80]. Similarly, duration of the convulsive disorder did not correlate with IQ scores computed post‐surgically.…”

“…It is, however, possible that the heterogeneity of the underlying neurological diseases reported in these various studies may have masked some of these relationships. In fact, in Ogunmekan et al 's study [76] of 10 hemispherectomized patients with Sturge‐Weber disease, a direct link was demonstrated between global IQ and duration of the convulsive disorder and/or age at surgery. Their sample was relatively homogenous in terms of seizure type (partial motor) and age of onset (8 weeks to 8 months).…”