Long-Term Follow-Up of Patients with BNS Epilepsy for More Than 30 to 40 Years: First Results

B. Fiedler; J. Krois-Neudenberger; J. Althaus; Y. Weber; G. Kurlemann

doi:10.1055/s-0034-1390563

Neuropediatrics, Table of Contents

Long-Term Follow-Up of Patients with BNS Epilepsy for More Than 30 to 40 Years: First Results

B. Fiedler ¹, J. Krois-Neudenberger ¹, J. Althaus ¹, Y. Weber ², G. Kurlemann ¹

¹Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Münster, Allgemeine Pädiatrie, Bereich Neuropädiatrie, Münster, Germany
²Hertie Institut für Klinische Hirnforschung, Universität Tübingen, Neurologie mit Schwerpunkt Epileptologie, Tübingen, Germany

Abstract

Blitz-Nick-Salaam (BNS) epilepsy (syn. West syndrome) is one of the severest forms of epilepsy generally involving impairments in child development which lead to permanent disability. In the literature, there are only a few studies of the long-term progression of the West syndrome and the development of affected children. Most follow-up periods described in these studies end before the affected children are 10 years old, and the majority of follow-ups do not exceed 60 months.

We examined a total of 447 (187 females and 260 male) former patients with West syndrome who were diagnosed between 1970 and 2010. Patients were assessed regarding the following: progression of epilepsy, therapy regimen, comorbidity, life quality, restrictions in daily life, depression scale, quality of sleep, socioeconomic status, school education, housing situation, driving license, severe disabilities, achieved motoric, and mental development levels.

We report on the first group in the period between 1970 and 1980. The group contains 51 patients, 34 with symptomatic and 17 with idiopathic West syndrome. Overall, 18 patients (35%) are deceased. In 10 cases, the cause of death is unclear, a direct connection to epilepsy existed in 2 cases (status epilepticus, death under adrenocorticotropic hormone therapy). Of the 33 surviving patients, 18 have consented to a clinical follow-up examination. Of these patients, three have a secondary school-leaving certificate and lead an independent life. One of the patients is now a practicing doctor. The remaining 15 patients were living in homes for handicapped or at home with their parents. They have either graduated from a special-needs school or have no school-leaving certificate at all. Of 18 patients, 9 still have persisting epileptic seizures. In the group with idiopathic West syndrome patients, their legal guardian agreed to a genetic follow-up examination in seven cases.

The long-term course of former West syndrome patients confirms the known poor prognosis for the further neurological development of the patients. In our small follow-up group, 15 of 18 patients do not lead independent lives. The high death rate of 35% during the observation study over 30 to 40 years is striking.