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Cryptogenic late-onset epileptic spasms

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Disease definition

Cryptogenic late-onset epileptic spasms is a rare epilepsy syndrome characterized by late-onset (after 1 year old) epileptic spasms that ocurr in clusters, associated with tonic seizures, atypical absences and cognitive deterioration. Language difficulties and behavior problems are frequently present. EEG is characterized by a temporal, or temporofrontal, slow wave or spike focus combined with synchronous spike-waves and no hypsarrhythmia or background activity.

ORPHA:163708

Classification level: Disorder

Synonym(s):
  • Late-onset infantile spasms

Prevalence: Unknown

Inheritance: -

Age of onset: Childhood

ICD-10: G40.4

ICD-11: 8A61.2Y

UMLS: C4755310

A summary on this disease is available in Français, Español, Italiano, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Español (2023) - Asociación Nacional de Personas con Epilepsia-ANPE
Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.