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 contribution||Early-onset progressive encephalopathy with migrant, continuous myoclonus|
|Number of pages||4|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - 1996|
ASJC Scopus subject areas
- Clinical Neurology