Encefalopatia progressiva a esordio precoce con mioclono migrante continuo

Translated title of the contribution: Early-onset progressive encephalopathy with migrant, continuous myoclonus

R. Gaggero, M. Baglietto, R. Devescovi, R. Curia, M. De Negri

Research output: Contribution to journalArticle


Four unusual cases of migrant continuous myoclonus with onset during the first months of life, are reported. During disease evolution, prolonged bilateral myoclonic and generalized tonic-clonic seizures occur, and a progressive encephalopathy with hypotonia and ataxia appear with a net neurological worsening after the age of 4-5 years. Cortical atrophy is shown by CCT and MRI. Neurometabolic screening is not contributory. Repeated polygrafic recordings show continuous and segmental myoclonic jerks in different muscles, at frequencies ranging between 0,5-1 c/s and 6-8 c/s; action myoclonus is recorded as well. This syndrome corresponds to an early onset progressive encephalopathy of unknown origin, similar in some aspects to Alper's disease.

Translated title of the contributionEarly-onset progressive encephalopathy with migrant, continuous myoclonus
Original languageItalian
Pages (from-to)499-502
Number of pages4
JournalBollettino - Lega Italiana contro l'Epilessia
Issue number95-96
Publication statusPublished - 1996

ASJC Scopus subject areas

  • Clinical Neurology

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