Document Detail

Myoclonus in Creutzfeldt-Jakob disease: polygraphic and video-electroencephalography assessment of 109 patients.
MedLine Citation:
PMID:  20939057     Owner:  NLM     Status:  In-Process    
We used electroencephalography (EEG)-polygraphic recordings to classify myoclonus in 109 patients with Creutzfeldt-Jakob disease (CJD) on the basis of its electromyography (EMG) pattern, time course, distribution, and EEG correlates. We recorded myoclonic jerks in 55 patients (50.4%), and we classified them as periodic myoclonus in 28, rhythmic in 13, and irregular in 20 (6 patients showed two types of myoclonus). Myoclonus occurred as a prominently negative event (interrupting the EMG discharge) in 10. Periodic sharp-wave complexes (PSWCs) were present in all but one patient with myoclonic jerks but were time-locked with EMG-bursts only in case of periodic myoclonus. Jerk-locked back averaging revealed a variable EEG-EMG transfer-time commonly exceeding that characterizing cortical myoclonus. Myoclonus was frequently associated with Met/Met polymorphism at codon 129 of the prion protein gene, but it was also observed in association with Met/Val or Val/Val polymorphisms provided that the EEG showed the presence of the PSWC pattern. The presence of enlarged somatosensory evoked potentials significantly correlated with the myoclonic presentation, as did MR signal hyperintensity involving the cortical mantle. Our observations on the basis of standard polygraphic criteria suggest that CJD associates with a remarkable variety of myoclonic jerks, and therefore different brain structures are probably involved as generators. The significant association between the presence of all myoclonus types with PSWCs suggests that hyperexcitable corticosubcortical loops are always required to generate (or allow) both myoclonus and the EEG complexes, either they are time locked or not.
Simona Binelli; Pamela Agazzi; Laura Canafoglia; Vidmer Scaioli; Ferruccio Panzica; Elisa Visani; Giuseppe Di Fede; Giorgio Giaccone; Alberto Bizzi; Orso Bugiani; Guiliano Avanzini; Fabrizio Tagliavini; Silvana Franceschetti
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  25     ISSN:  1531-8257     ISO Abbreviation:  Mov. Disord.     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2818-27     Citation Subset:  IM    
Copyright Information:
© 2010 Movement Disorder Society.
Unit of Neurophysiopathology, IRCCS Foundation C. Besta Neurological Institute, Milan, Italy.
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