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Progressive myoclonic epilepsy type 3

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Disease definition

A rare epilepsy syndrome characterized by infantile- to early childhood-onset of progressive myoclonic seizures (occasionally accompanied by generalized tonic-clonic seizures) and severe, progressive neurological regression, leading to psychomotor and cognitive decline, cerebellar ataxia, dementia and, frequently, early death. Vision loss may be associated. EEG typically reveals epileptiform activity with predominance in the posterior region and photosensitivity.

ORPHA:263516

Classification level: Disorder

Synonym(s):
  • EPM3
  • PME type 3
  • Progressive myoclonic epilepsy due to KCTD7 deficiency
  • Progressive myoclonus epilepsy type 3

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Childhood, Infancy

ICD-10: G40.3

ICD-11: 8A61.41

OMIM: 611726

UMLS: C2673257

MeSH: C567095

GARD: 2167

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
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 (2013.pdf) - Orphanet Urgences
Anesthesia guidelines
English (2016) - Orphananesthesia
Čeština (2016) - Orphananesthesia
Disease review articles
Review article
English (2016) - Orphanet J Rare Dis
Clinical genetics review
English (2013) - GeneReviews
Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
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.