MovDisordV3, Main, Exploration, bibRecord, 001B66

Myoclonus in Creutzfeldt‐Jakob disease: Polygraphic and video‐electroencephalography assessment of 109 patients

Identifieur interne : 001B66 ( Main/Exploration ); précédent : 001B65; suivant : 001B67

Myoclonus in Creutzfeldt‐Jakob disease: Polygraphic and video‐electroencephalography assessment of 109 patients

Auteurs : Simona Binelli [Italie] ; Pamela Agazzi [Italie] ; Laura Canafoglia [Italie] ; Vidmer Scaioli [Italie] ; Ferruccio Panzica [Italie] ; Elisa Visani [Italie] ; Giuseppe Di Fede [Italie] ; Giorgio Giaccone [Italie] ; Alberto Bizzi [Italie] ; Orso Bugiani [Italie] ; Guiliano Avanzini [Italie] ; Fabrizio Tagliavini [Italie] ; Silvana Franceschetti [Italie]

Source :

Movement Disorders [ 0885-3185 ] ; 2010-12-15.

RBID : ISTEX:D4C83E2F930F2286B581FF65E76D43CAAF79FBD8

Descripteurs français

Pascal (Inist)
- Electroencéphalographie, Electromyographie, Encéphalopathie spongiforme de Creutzfeldt-Jakob, Homme, Myoclonie, Pathologie du système nerveux, Polygraphie, Prion.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Aged, Analysis of Variance, Brain (physiopathology), Creutzfeldt-Jakob Syndrome (complications), Creutzfeldt-Jakob Syndrome (physiopathology), Creutzfeldt-Jakob disease, Creutzfeldt‐Jakob, EEG‐EMG relationship, Electroencephalography, Electromyography, Human, Humans, Male, Middle Aged, Myoclonus, Myoclonus (classification), Myoclonus (complications), Myoclonus (diagnosis), Myoclonus (physiopathology), Nervous system diseases, Polygraphy, Prion, Videotape Recording, back‐averaging, myoclonus, polygraphy.
MESH :
- classification : Myoclonus.
- complications : Creutzfeldt-Jakob Syndrome, Myoclonus.
- diagnosis : Myoclonus.
- physiopathology : Brain, Creutzfeldt-Jakob Syndrome, Myoclonus.
- Aged, Analysis of Variance, Electroencephalography, Electromyography, Humans, Male, Middle Aged, Videotape Recording.

Abstract

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. © 2010 Movement Disorder Society

Url:

https://api.istex.fr/document/D4C83E2F930F2286B581FF65E76D43CAAF79FBD8/fulltext/pdf

DOI: 10.1002/mds.23397

Affiliations:

Le document en format XML

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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Aged</term>
<term>Analysis of Variance</term>
<term>Brain (physiopathology)</term>
<term>Creutzfeldt-Jakob Syndrome (complications)</term>
<term>Creutzfeldt-Jakob Syndrome (physiopathology)</term>
<term>Creutzfeldt-Jakob disease</term>
<term>Creutzfeldt‐Jakob</term>
<term>EEG‐EMG relationship</term>
<term>Electroencephalography</term>
<term>Electromyography</term>
<term>Human</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Myoclonus</term>
<term>Myoclonus (classification)</term>
<term>Myoclonus (complications)</term>
<term>Myoclonus (diagnosis)</term>
<term>Myoclonus (physiopathology)</term>
<term>Nervous system diseases</term>
<term>Polygraphy</term>
<term>Prion</term>
<term>Videotape Recording</term>
<term>back‐averaging</term>
<term>myoclonus</term>
<term>polygraphy</term>
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<keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Myoclonus</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Creutzfeldt-Jakob Syndrome</term>
<term>Myoclonus</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Myoclonus</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain</term>
<term>Creutzfeldt-Jakob Syndrome</term>
<term>Myoclonus</term>
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<keywords scheme="MESH" xml:lang="en"><term>Aged</term>
<term>Analysis of Variance</term>
<term>Electroencephalography</term>
<term>Electromyography</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Videotape Recording</term>
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<term>Electromyographie</term>
<term>Encéphalopathie spongiforme de Creutzfeldt-Jakob</term>
<term>Homme</term>
<term>Myoclonie</term>
<term>Pathologie du   système nerveux</term>
<term>Polygraphie</term>
<term>Prion</term>
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<front><div type="abstract" xml:lang="en">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. © 2010 Movement Disorder Society</div>
</front>
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<name sortKey="Agazzi, Pamela" sort="Agazzi, Pamela" uniqKey="Agazzi P" first="Pamela" last="Agazzi">Pamela Agazzi</name>
<name sortKey="Avanzini, Guiliano" sort="Avanzini, Guiliano" uniqKey="Avanzini G" first="Guiliano" last="Avanzini">Guiliano Avanzini</name>
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<name sortKey="Franceschetti, Silvana" sort="Franceschetti, Silvana" uniqKey="Franceschetti S" first="Silvana" last="Franceschetti">Silvana Franceschetti</name>
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<name sortKey="Visani, Elisa" sort="Visani, Elisa" uniqKey="Visani E" first="Elisa" last="Visani">Elisa Visani</name>
</country>
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Movement Disorders (revue)

Myoclonus in Creutzfeldt‐Jakob disease: Polygraphic and video‐electroencephalography assessment of 109 patients

Myoclonus in Creutzfeldt‐Jakob disease: Polygraphic and video‐electroencephalography assessment of 109 patients

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Abstract

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	Movement Disorders (revue)
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