Myoclonus generators in sialidosis

ObjectiveSialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of...

Full description

Saved in:
Bibliographic Details
Published in:Clinical neurophysiology practice 2022, Vol.7, p.169-173
Main Authors: Vial, Felipe, McGurrin, Patrick, Attaripour, Sanaz, d'Azzo, Alesandra, Tifft, Cynthia J, Toro, Camilo, Hallett, Mark
Format: Report
Language:English
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
cited_by
cites
container_end_page 173
container_issue
container_start_page 169
container_title Clinical neurophysiology practice
container_volume 7
creator Vial, Felipe
McGurrin, Patrick
Attaripour, Sanaz
d'Azzo, Alesandra
Tifft, Cynthia J
Toro, Camilo
Hallett, Mark
description ObjectiveSialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of a subcortical basis for synchronous myoclonic phenomena. MethodsElectromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1. ResultsThe EMG recordings showed clear episodes of bilaterally synchronous myoclonic activity in contralateral homologous muscles. We also observed a high muscular-muscular coherence with near-zero time-lag between these muscles. ConclusionThe absence of coherence phase lag between the right-and-left homologous muscles during synchronous events indicates that a unilateral cortical source cannot fully explain the myoclonic activity. There must exist a subcortical mechanism for bilateral synchronization accounting for this phenomenon. SignificanceUnderstanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.
doi_str_mv 10.1016/j.cnp.2022.05.004
format report
fullrecord <record><control><sourceid>proquest</sourceid><recordid>TN_cdi_proquest_miscellaneous_2687721112</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2687721112</sourcerecordid><originalsourceid>FETCH-proquest_miscellaneous_26877211123</originalsourceid><addsrcrecordid>eNqVyrEKwjAQgOFDECzaB3ArTi7Gu7M2dRbFxc3BrYQaJSUmtdcOvr0IvoDL_y0_wJxQEVKxblQdWsXIrHCrEPMRJJwXerUr6TqBVKRBRNL4bQKL8zvWPoZBsocNtjN97CRzIRNnvLtFcTKD8d14senPKSyPh8v-tGq7-Bqs9NXTSW29N8HGQSouSq2ZiHjzx_oB0HA4VQ</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>report</recordtype><pqid>2687721112</pqid></control><display><type>report</type><title>Myoclonus generators in sialidosis</title><source>ScienceDirect Journals</source><source>PubMed Central</source><creator>Vial, Felipe ; McGurrin, Patrick ; Attaripour, Sanaz ; d'Azzo, Alesandra ; Tifft, Cynthia J ; Toro, Camilo ; Hallett, Mark</creator><creatorcontrib>Vial, Felipe ; McGurrin, Patrick ; Attaripour, Sanaz ; d'Azzo, Alesandra ; Tifft, Cynthia J ; Toro, Camilo ; Hallett, Mark</creatorcontrib><description>ObjectiveSialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of a subcortical basis for synchronous myoclonic phenomena. MethodsElectromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1. ResultsThe EMG recordings showed clear episodes of bilaterally synchronous myoclonic activity in contralateral homologous muscles. We also observed a high muscular-muscular coherence with near-zero time-lag between these muscles. ConclusionThe absence of coherence phase lag between the right-and-left homologous muscles during synchronous events indicates that a unilateral cortical source cannot fully explain the myoclonic activity. There must exist a subcortical mechanism for bilateral synchronization accounting for this phenomenon. SignificanceUnderstanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.</description><identifier>EISSN: 2467-981X</identifier><identifier>DOI: 10.1016/j.cnp.2022.05.004</identifier><language>eng</language><ispartof>Clinical neurophysiology practice, 2022, Vol.7, p.169-173</ispartof><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>776,780,4476,27902</link.rule.ids></links><search><creatorcontrib>Vial, Felipe</creatorcontrib><creatorcontrib>McGurrin, Patrick</creatorcontrib><creatorcontrib>Attaripour, Sanaz</creatorcontrib><creatorcontrib>d'Azzo, Alesandra</creatorcontrib><creatorcontrib>Tifft, Cynthia J</creatorcontrib><creatorcontrib>Toro, Camilo</creatorcontrib><creatorcontrib>Hallett, Mark</creatorcontrib><title>Myoclonus generators in sialidosis</title><title>Clinical neurophysiology practice</title><description>ObjectiveSialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of a subcortical basis for synchronous myoclonic phenomena. MethodsElectromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1. ResultsThe EMG recordings showed clear episodes of bilaterally synchronous myoclonic activity in contralateral homologous muscles. We also observed a high muscular-muscular coherence with near-zero time-lag between these muscles. ConclusionThe absence of coherence phase lag between the right-and-left homologous muscles during synchronous events indicates that a unilateral cortical source cannot fully explain the myoclonic activity. There must exist a subcortical mechanism for bilateral synchronization accounting for this phenomenon. SignificanceUnderstanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.</description><issn>2467-981X</issn><fulltext>true</fulltext><rsrctype>report</rsrctype><creationdate>2022</creationdate><recordtype>report</recordtype><recordid>eNqVyrEKwjAQgOFDECzaB3ArTi7Gu7M2dRbFxc3BrYQaJSUmtdcOvr0IvoDL_y0_wJxQEVKxblQdWsXIrHCrEPMRJJwXerUr6TqBVKRBRNL4bQKL8zvWPoZBsocNtjN97CRzIRNnvLtFcTKD8d14senPKSyPh8v-tGq7-Bqs9NXTSW29N8HGQSouSq2ZiHjzx_oB0HA4VQ</recordid><startdate>20220101</startdate><enddate>20220101</enddate><creator>Vial, Felipe</creator><creator>McGurrin, Patrick</creator><creator>Attaripour, Sanaz</creator><creator>d'Azzo, Alesandra</creator><creator>Tifft, Cynthia J</creator><creator>Toro, Camilo</creator><creator>Hallett, Mark</creator><scope>7X8</scope></search><sort><creationdate>20220101</creationdate><title>Myoclonus generators in sialidosis</title><author>Vial, Felipe ; McGurrin, Patrick ; Attaripour, Sanaz ; d'Azzo, Alesandra ; Tifft, Cynthia J ; Toro, Camilo ; Hallett, Mark</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-proquest_miscellaneous_26877211123</frbrgroupid><rsrctype>reports</rsrctype><prefilter>reports</prefilter><language>eng</language><creationdate>2022</creationdate><toplevel>online_resources</toplevel><creatorcontrib>Vial, Felipe</creatorcontrib><creatorcontrib>McGurrin, Patrick</creatorcontrib><creatorcontrib>Attaripour, Sanaz</creatorcontrib><creatorcontrib>d'Azzo, Alesandra</creatorcontrib><creatorcontrib>Tifft, Cynthia J</creatorcontrib><creatorcontrib>Toro, Camilo</creatorcontrib><creatorcontrib>Hallett, Mark</creatorcontrib><collection>MEDLINE - Academic</collection></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vial, Felipe</au><au>McGurrin, Patrick</au><au>Attaripour, Sanaz</au><au>d'Azzo, Alesandra</au><au>Tifft, Cynthia J</au><au>Toro, Camilo</au><au>Hallett, Mark</au><format>book</format><genre>unknown</genre><ristype>RPRT</ristype><atitle>Myoclonus generators in sialidosis</atitle><jtitle>Clinical neurophysiology practice</jtitle><date>2022-01-01</date><risdate>2022</risdate><volume>7</volume><spage>169</spage><epage>173</epage><pages>169-173</pages><eissn>2467-981X</eissn><abstract>ObjectiveSialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of a subcortical basis for synchronous myoclonic phenomena. MethodsElectromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1. ResultsThe EMG recordings showed clear episodes of bilaterally synchronous myoclonic activity in contralateral homologous muscles. We also observed a high muscular-muscular coherence with near-zero time-lag between these muscles. ConclusionThe absence of coherence phase lag between the right-and-left homologous muscles during synchronous events indicates that a unilateral cortical source cannot fully explain the myoclonic activity. There must exist a subcortical mechanism for bilateral synchronization accounting for this phenomenon. SignificanceUnderstanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.</abstract><doi>10.1016/j.cnp.2022.05.004</doi></addata></record>
fulltext fulltext
identifier EISSN: 2467-981X
ispartof Clinical neurophysiology practice, 2022, Vol.7, p.169-173
issn 2467-981X
language eng
recordid cdi_proquest_miscellaneous_2687721112
source ScienceDirect Journals; PubMed Central
title Myoclonus generators in sialidosis
url http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-14T22%3A36%3A32IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest&rft_val_fmt=info:ofi/fmt:kev:mtx:book&rft.genre=unknown&rft.atitle=Myoclonus%20generators%20in%20sialidosis&rft.jtitle=Clinical%20neurophysiology%20practice&rft.au=Vial,%20Felipe&rft.date=2022-01-01&rft.volume=7&rft.spage=169&rft.epage=173&rft.pages=169-173&rft.eissn=2467-981X&rft_id=info:doi/10.1016/j.cnp.2022.05.004&rft_dat=%3Cproquest%3E2687721112%3C/proquest%3E%3Cgrp_id%3Ecdi_FETCH-proquest_miscellaneous_26877211123%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=2687721112&rft_id=info:pmid/&rfr_iscdi=true