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Epilepsy syndromes information


An epilepsy syndrome is defined as "a characteristic cluster of clinical and EEG features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)."[1]

Syndromes are characterized by certain seizure type(s) and specific findings on EEG. Epilepsy syndromes often begin, and may remit, at specific ages. Identification of an epilepsy syndrome may provide important clues to the likely cause, the most effective treatment and the risk of comorbidities such as learning problems, intellectual disability, ADHD or other problems.[1]

Not everyone with epilepsy can be defined as having an epilepsy syndrome.[1] Epilepsy syndromes are most commonly found in children with epilepsy onset before 3 years of age and are less common in adult-onset epilepsy.[2]

This article reflects the 2017 ILAE Classification of the Epilepsies, and its more detailed follow-up papers, produced for the International League Against Epilepsy by a number of specialist clinicians.[3] Some syndromes in earlier classifications have been renamed or redefined, but these are retained at the end of the article for convenience.

  1. ^ a b c Wirrell EC, Nabbout R, Scheffer IE, Alsaadi T, Bogacz A, French JA, 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. https://doi.org/10.1111/epi.17237
  2. ^ Neligan, A; Hauser, WA; Sander, JW (2012). "The epidemiology of the epilepsies". Handbook of Clinical Neurology. 107: 113–33. doi:10.1016/B978-0-444-52898-8.00006-9. ISBN 9780444528988. PMID 22938966.
  3. ^ [https://www.ilae.org/guidelines/definition-and-classification/classification-and-definition-of-epilepsy-syndromes "Classification and Definition of Epilepsy Syndromes", ILAE

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Epilepsy syndromes

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various seizure syndromes, including myoclonic epilepsy, familial neonatal convulsions, childhood absence epilepsy, absence epilepsy, infantile spasms...

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Dravet syndrome

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List of syndromes

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of medical syndromes. 13q deletion syndrome Cordoba Syndrome 17q21.31 microdeletion syndrome 1p36 deletion syndrome 1q21.1 deletion syndrome 1q21.1 duplication...

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Absence seizure

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epilepsy. Other proposed syndromes are Jeavons syndrome (eyelid myoclonia with absences), and genetic generalised epilepsy with phantom absences. Absence...

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Lamotrigine

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absence epilepsy and other generalised epilepsy syndromes, including primary generalised tonic–clonic seizures, juvenile myoclonic epilepsy, and Lennox-Gastaut...

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Epileptic spasms

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that caused by premature birth. West syndrome appears in 1% to 5% of infants with Down syndrome. This form of epilepsy is relatively difficult to treat in...

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MERRF syndrome

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Panayiotopoulos syndrome

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Minassian, BA. (Jul 2009). "Treatment options for epileptic myoclonus and epilepsy syndromes associated with myoclonus". Expert Opin Pharmacother. 10 (10): 1549–60...

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Reflex seizure

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myoclonic, or absence seizures. Epilepsy syndromes characterized by repeated reflex seizures are known as reflex epilepsies. Photosensitive seizures are...

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Approximately 60% of all people with epilepsy (0.4% of the population of industrialized countries) have focal epilepsy syndromes. In 15% to 20% of these patients...

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Jeavons syndrome

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the third condition that is a diagnosed epilepsy syndrome. Epilepsy syndromes are notably different than epilepsy types, they are identified by a combination...

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Juvenile myoclonic epilepsy

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Sudden unexpected death in epilepsy

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Myoclonic epilepsy

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activity. There are two syndromes and several related disorders. Juvenile myoclonic epilepsy is responsible for 7% of cases of epilepsy. Seizures usually begin...

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Geschwind syndrome

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Ketogenic diet

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Ingrid Scheffer

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Beyond further describing the aetiology of epilepsy, Ingrid has worked to characterise new epilepsy syndromes, from infancy to adulthood, which have permitted...

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Northern epilepsy syndrome

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Northern epilepsy syndrome (NE), or progressive epilepsy with mental retardation (EPMR), is a subtype of neuronal ceroid lipofuscinosis and a rare disease...

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Rage syndrome

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limbic epilepsy for rage syndrome has been used synonymously with the terms psychomotor epilepsy, automatic epilepsy, rhinencephalic epilepsy, behavioral...

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Seizure

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ages of six months and five years. During childhood, well-defined epilepsy syndromes are generally seen. In adolescence and young adulthood, non-compliance...

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Ramsay Hunt syndrome

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Three different neurological syndromes carry the name of Ramsay Hunt syndrome. Their only connection is that they were all first documented by the famous...

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