Specific Epileptic Syndromes Are Rare Even in Tertiary Epilepsy Centers: A Patient‐oriented Approach to Epilepsy Classification

Abstract

Summary:Purpose: To assess the practicability and reliability of a five-dimensional patient-oriented epilepsy classification and to compare it with the International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The dimensions consist of the epileptogenic zone, semiologic seizure type(s), etiology, related medical conditions, and seizure frequency.Methods: The 185 epilepsy patients (94 adults, 91 children, aged 18 years or younger) were randomly selected from the database of a tertiary epilepsy center and the general neurological department of a metropolitan hospital (28 adults). The charts were reviewed independently by two investigators and classified according to both the ILAE and the patient-oriented classification. Interrater reliability was assessed, and a final consensus among all investigators was established.Results: Only four (4%) adults and 19 (21%) children were diagnosed with a specific epilepsy syndrome of the ILAE classification. All other patients were in unspecific categories. The patient-oriented classification revealed that 64 adults and 56 children had focal epilepsy. In an additional 34 adults and 45 children, the epileptogenic zone could be localized to a certain brain region, and in 14 adults and five children, the epileptogenic zone could be lateralized. Fourteen adults and 21 children had generalized epilepsy. In 16 adults and 14 children, it remained unclear whether the epilepsy was focal or generalized. Generalized simple motor seizures were found in 66 adults and 52 children, representing the most frequent seizure type. Etiology could be determined in 40 adults and 45 children. Hippocampal sclerosis was the most frequent etiology in adults (10%), and cortical dysplasia (9%), in children. Seven adults and 31 children had at least daily seizures. Seventeen adults and 26 children had rare or no seizures at their last documented contact. The most frequent related medical conditions were psychiatric disorders and mental retardation. Interrater agreement was high (kappa values of 0.8 to 0.9) for both the patient-oriented and the ILAE classification.Conclusions: Specific epilepsy syndromes included in the current ILAE classification are rare even in a tertiary epilepsy center. Most patients are included in unspecific categories that provide only incomplete information. In contrast, all of the patients could be classified by the five-dimensional patient-oriented classification, providing all essential information for the management of the patients with a high degree of interrater reliability.

Autistic characteristics in adults with epilepsy

“…Kellinghaus attempts to directly address the reliability of ILAE epilepsy classification (Kellinghaus et al, 2004). Kellinghaus and colleagues classified 185 epilepsy patients, and found that only four 4% of adults were diagnosed with a specific epilepsy syndrome of the ILAE classification.…”

“…Frequent seizures in childhood may lead to irreversible connectivity changes in important neuronal pathways (Kellinghaus et al, 2004).…”

“…Frequent seizures in childhood may lead to irreversible connectivity changes in important neuronal pathways (Kellinghaus et al, 2004). Importantly, early-life seizure activity has been related to unrecoverable damage to the structure and functioning of the PFC and inflexible behaviour (Kleen et al, 2011).…”

Vorschlag für eine neue patientenorientierte Epilepsieklassifikation

“…In nichtspezialisierten neurologischen Praxen beträgt der Anteil lokalisationsbezogener Epilepsien bei 0,7, und der Anteil definierter generalisierter Syndrome bei 10 [2]. Selbst in einem tertiären Epilepsiezentrum in den USA konnte bei lediglich 4 der Erwachsenen und bei 19 der pädiatrischen Patienten ein spezielles ILAE-Syndrom diagnostiziert werden [11]. Diese Studien zeigen, dass das von der ILAE-Arbeitsgruppe vorgeschlagene Konzept lediglich auf eine Minderheit der Epilepsiepatienten anwendbar ist.…”

Vorschlag für eine neue patientenorientierte Epilepsieklassifikation*

“…In nichtspezialisierten neurologischen Praxen beträgt der Anteil lokalisationsbezogener Epilepsien bei 0,7%, und der Anteil definierter generalisierter Syndrome bei 10% [2]. Selbst in einem tertiären Epilepsiezentrum in den USA konnte bei lediglich 4% der Erwachsenen und bei 19% der pädiatrischen Patienten ein spezielles ILAESyndrom diagnostiziert werden [11]. Diese Studien zeigen, dass das von der ILAE-Arbeitsgruppe vorgeschlagene Konzept lediglich auf eine Minderheit der Epilepsiepatienten anwendbar ist.…”