Early-onset progressive encephalopathy with migrant continuous myoclonus

What is Early-onset progressive encephalopathy with migrant continuous myoclonus?

A rare infantile epilepsy syndrome characterized by early-onset progressive encephalopathy with migrant, continuous myoclonus. The focal continuous myoclonus (lasting from dozens of minutes to hours) is observed in the first months of life. During disease progression, prolonged bilateral myoclonic seizures and generalized tonic-clonic seizures occur and eventually progressive encephalopathy with hypotonia and ataxia is observed. All patients reported to have cortical atrophy. There have been no further descriptions in the literature since 1996.

Inheritance type

Unknown

Prevalence

  • Worldwide: <1 in 1,000 000

Age of Onset

  • Childhood
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.