Homepage > Rare diseases > Search

Search for a rare disease

*
(*) mandatory field

Progressive myoclonic epilepsy type 6

Suggest an update
Your message has been sent Your message has not been sent. Please contact an administrator.
Disease definition

A rare, genetic, neurological disorder characterized by early-onset, progressive ataxia associated with myoclonic seizures (frequently associated with other seizure types such as generalized tonic-clonic, absence and drop attacks), scoliosis of variable severity, areflexia, elevated creatine kinase serum levels, and relative preservation of cognitive function until late in the disease course.

ORPHA:280620

Classification level: Disorder

Synonym(s):
  • EPM6
  • GOSR2-related progressive myoclonus ataxia
  • North Sea progressive myoclonus epilepsy
  • PME type 6
  • Progressive myoclonus epilepsy type 6

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Childhood

ICD-10: G40.3

ICD-11: 8A61.41

OMIM: 614018

UMLS: C5190805

GARD: 3872

A summary on this disease is available in Français, Español, 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
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.