| Non-epileptic myoclonus and mitochondrial encephalomyopathy. | |
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MedLine Citation:
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PMID: 2619613 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Two brothers presented to us with a progressive myoclonic syndrome with slight cerebellar symptoms. Neurological examination disclosed moderate cerebellar signs and pale optic discs; asymmetric, asynchronous and arrhythmic myoclonus, an arrthesthesic deficit and no muscular weakness. EEG background activity was moderately slow with no irritative discharges. CT was normal in both cases. Intermittent photic stimulation increased the frequency of the myoclonic jerks, which became bilateral and synchronous, progressing to a generalized tonic-clonic seizure. EPs and MRI in one case were normal. Anticonvulsant drugs were ineffective. The diagnosis of mitochondrial encephalomyopathy was based on the finding, in muscle specimens, of thickened basement membranes with myofibrillary degeneration and increased number of mitochondria peripherally distributed and with a dense granular matrix and some vacuoles. The clinical and EEG data suggest a subcortical origin for this type of myoclonic syndrome. |
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Authors:
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A Cukiert; F G Naylor; H B Scapolan; M M Vilela; F S Aloe; J O Siffert; A M Tsanaclis; M Haddad; T C Machado; M Carvalho-Alegro |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Arquivos de neuro-psiquiatria Volume: 47 ISSN: 0004-282X ISO Abbreviation: Arq Neuropsiquiatr Publication Date: 1989 Sep |
Date Detail:
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Created Date: 1990-03-15 Completed Date: 1990-03-15 Revised Date: 2009-11-11 |
Medline Journal Info:
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Nlm Unique ID: 0125444 Medline TA: Arq Neuropsiquiatr Country: BRAZIL |
Other Details:
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Languages: eng Pagination: 346-51 Citation Subset: IM |
Affiliation:
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Faculty of Medicine, University of São Paulo, Brasil. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Brain Diseases / physiopathology* Cerebral Cortex / physiopathology Electroencephalography Evoked Potentials Humans Male Middle Aged Mitochondria, Muscle / ultrastructure* Myoclonus / diagnosis*, etiology Neuromuscular Diseases / pathology* |
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