Early-onset progressive encephalopathy with migrant, continuous myoclonus

Roberto Gaggero, Maria Pia Baglietto, Roberto Curia, Maurizio De Negri

Research output: Contribution to journalArticlepeer-review

Abstract

Three unusual cases of focal continuous myoclonus with onset during the first months of life, lasting from dozens of minutes to hours, are reported. During disease evolution, prolonged bilateral myoclonic seizures and generalized tonic-clonic seizures occur. Subsequently, a progressive encephalopathy with hypotonia and ataxia appears. A net worsening of the neurological condition is observed after the age of 4-5 years. Cortical atrophy is shown by CCT and MRI. Neurometabolic screening is not contributory. Repeated polygraphic recordings show continuous and segmental myoclonic jerks, localized in different muscles, at frequencies ranging between 0.5-1 c/s and 6-8 c/s. Moreover action myoclonus is recorded. During the first period of disease the EEG does not show any paroxysmal activity. As to the classification, this syndrome corresponds to an early onset progressive encephalopathy of unknown origin, similar in some aspects to Alper's disease. Another problem is the interpretation of the myoclonic phenomena. Some important aspects suggest a cortical origin of the diverse myoclonic phenomena observed in these cases.

Original languageEnglish
Pages (from-to)254-261
Number of pages8
JournalChild's Nervous System
Volume12
Issue number5
Publication statusPublished - May 1996

Keywords

  • Alper's disease
  • Epilepsia partialis continua
  • Focal myoclonus
  • Progressive encephalopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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