Klassifikation von Epilepsiesyndromen

Im Jahr 2022 wurden von der Nosology and Definitions Task Force der Internationalen Liga gegen Epilepsie (ILAE) in einer Serie von Positionspapieren erstmals von der ILAE anerkannte Definitionen von Epilepsiesyndromen vorgestellt, die in der vorliegenden Übersichtsarbeit kurz zusammengefasst werden sollen [1–6].

Abstract

Epilepsy syndromes are defined as a characteristic cluster of clinical and EEG features, often supported by specific etiologic findings (structural, genetic, metabolic, immune or infectious). Epilepsy syndromes are grouped according to the age at epilepsy onset in epilepsy syndromes with neonatal and infantile onset, epilepsy syndromes with childhood onset and epilepsy syndromes with variable age at onset. According to seizure types in each age group syndromes are further subdivided into generalized, focal or combined focal and generalized. Developmental and epileptic encephalopathies (DEE) and syndromes with progressive neurological deterioration represent an additional separate category in each age group. Idiopathic generalized epilepsies are combined in a separate group irrespective of age at onset. For each syndrome the following information is summarized: epidemiology, clinical context, natural history, seizure types, EEG, neuroimaging and genetic findings, other relevant laboratory studies and differential diagnosis. For each syndrome mandatory and exclusionary criteria as well as alerts are presented. The term syndrome in evolution should be used for syndromes in which some mandatory signs are still missing early in the epilepsy course, but appear with further disease progression. Etiology-specific syndromes are defined as syndromes in which a specific etiology is associated with a clearly defined, relatively uniform and distinct clinical phenotype as well as with consistent findings on EEG, neuroimaging and genetics in most affected individuals.

Schlüsselwörter

Anfallsformen - EEG - Epilepsiebeginn - Ätiologie

Key word

seizure type - EEG - epilepsy onset - etiology

muscle cramps - spasm - electromyography

Publication History

Article published online:
07 September 2023

• Literatur

• 1 Wirrell E, Tinuper P, Perucca E. et al. Introduction to the epilepsy syndrome papers. Epilepsia 2022; 63: 1330-1332 DOI: 10.1111/epi.17262.
• 2 Wirrell EC, Nabbout R, Scheffer IE. et al. Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63: 1333-1348 DOI: 10.1111/epi.17237.
• 3 Zuberi SM, Wirrell E, Yozawitz E. et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63: 1349-1397 DOI: 10.1111/epi.17239.
• 4 Specchio N, Wirrell EC, Scheffer IE. et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63: 1398-1442 DOI: 10.1111/epi.17241.
• 5 Riney K, Bogacz A, Somerville E. et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63: 1443-1474 DOI: 10.1111/epi.17240.
• 6 Hirsch E, French J, Scheffer IE. et al. ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63: 1475-1499 DOI: 10.1111/epi.17236.
• 7 [Anonym]. Proposal for classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1985; 26: 268-278
• 8 Tissot DM. Traité de l’épilepsie. Faisant le tonne troisième du traité des nerf & de leur maladies. Paris: Didot P.F., le jeune; 1770
• 9 Sauer H. Über gehäufte kleine Anfälle bei Kindern (Pyknolepsie). Psychiatr Neurol 1916; 40: 276-300
• 10 Adie WJ. Pyknolepsy; A form of Epilepsy in Children, with a good Prognosis. Proc R Soc Med 1924; 17: 19-25
• 11 Janz D. Die klinische Stellung der Pyknolepsie. Dtsch Med Wochenschr 1955; 80: 1392-1400 DOI: 10.1055/s-0028-1116209.
• 12 West WJ. On a peculiar form of infantile convulsions. Lancet 1841; 35: 724-725
• 13 Gibbs FA, Gibbs EL. Atlas of Electroencephalography. Cambridge, MA: Addison Wesley; 1952
• 14 Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 1950; 5: 626-644
• 15 Gastaut H, Roger J, Soulayrol R. et al. Epileptic encephalopathy of children with diffuse slow spikes and waves (alias “petit mal variant”) or Lennox syndrome. Ann Pediatr (Paris) 1966; 13: 489-499
• 16 Roger J, Dravet C, Bureau M. et al. Epileptic syndromes in childhood and adolescence. London, UK: John Libbey Eurotext; 1984
• 17 Bureau M, Genton P, Delgado-Escueta AV. et al. Epileptic Syndromes in Infancy, Childhood and Adolescence. 6th ed. Arcueil: John Libbey Eurotext; 2019
• 18 [Anonym]. Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League against Epilepsy. Epilepsia 1989; 30: 389-399
• 19 Scheffer IE, Berkovic S, Capovilla G. et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017; 58: 512-521 DOI: 10.1111/epi.13709.
• 20 Scheffer IE, Berkovic S, Capovilla G. et al. ILAE-Klassifikation der Epilepsien: Positionspapier der ILAE-Kommission für Klassifikation und Terminologie. Z Epileptol 2018; 31: 296-306
• 21 Cavazzuti GB, Cappella L, Nalin A. Longitudinal study of epileptiform EEG patterns in normal children. Epilepsia 1980; 21: 43-55 DOI: 10.1111/j.1528-1157.1980.tb04043.x.
• 22 Eeg-Olofsson O, Petersén I, Selldén U. The development of the electroencephalogram in normal children from the age of 1 through 15 years. Paroxysmal activity. Neuropadiatrie 1971; 2: 375-404 DOI: 10.1055/s-0028-1091791.
• 23 Sam MC, So EL. Significance of epileptiform discharges in patients without epilepsy in the community. Epilepsia 2001; 42: 1273-1278 DOI: 10.1046/j.1528-1157.2001.17101.x.
• 24 Berg AT, Berkovic SF, Brodie MJ. et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010; 51: 676-685
• 25 Berg AT, Berkovic SF, Brodie MJ. et al. Revidierte Terminologie und Konzepte zur Einteilung von epileptischen Anfällen und Epilepsien: Bericht der Klassifikations- und Terminologiekommission der Internationalen Liga gegen Epilepsie, 2005–2009. Akt Neurol 2010; 37: 120-130
• 26 Steriade C, Britton J, Dale RC. et al. Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune-associated epilepsy: Conceptual definitions. Epilepsia 2020; 61: 1341-1351 DOI: 10.1111/epi.16571.