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idiopathic generalized epilepsy

Includes the syndromes: - juvenile absence epilepsy - juvenile myoclonic epilepsy - epilepsy with only generalized tonic-clonic seizures Etiology: - genetic - type 6 is polygenic & multifactorial Epidemiology: - 1/3 of all epilepsy syndromes Genetics: - associated with defects in CACNA1H (type 6) - associated with defects in CACNB4 Clinical manifestations: - recurring generalized seizures in the absence of detectable brain lesions &/or metabolic abnormalities - generalized seizures arise diffusely & simultaneously from both hemispheres of the brain - tonic-clonic seizures, absence seizures or myoclonic seizures - cognitive deficits [3] - impulsivity - personality disorder - psychiatric disorder - may be present 5 years prior to diagnosis [3] Special laboratory: - electroencephalogram may show generalised spike wave abnormality [2] Radiology: - MRI neuroimaging generally normal [2]

Specific

epilepsy with grand mal seizures on awakening; epilepsy with only generalized tonic-clonic seizures juvenile absence epilepsy juvenile myoclonic epilepsy (of Janz)

General

epilepsy

Database Correlations

OMIM 611942

References

  1. OMIM :accession 611942
  2. Medical Knowledge Self Assessment Program (MKSAP) 17, American College of Physicians, Philadelphia 2015 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Gesche J, Rubboli G, Beier CP. Prodromal phase of idiopathic generalized epilepsy: A register-based case control study. Neurology 2024 Oct 22; 103:e209921. PMID: 39284111 https://www.neurology.org/doi/10.1212/WNL.0000000000209921