Felipe Vial , Patrick McGurrin , Sanaz Attaripour , Alesandra d'Azzo , Cynthia J. Tifft , Camilo Toro , Mark Hallett
{"title":"唾液中毒的肌阵挛产生者","authors":"Felipe Vial , Patrick McGurrin , Sanaz Attaripour , Alesandra d'Azzo , Cynthia J. Tifft , Camilo Toro , Mark Hallett","doi":"10.1016/j.cnp.2022.05.004","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><p>Sialidosis 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.</p></div><div><h3>Methods</h3><p>Electromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1.</p></div><div><h3>Results</h3><p>The 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.</p></div><div><h3>Conclusion</h3><p>The 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.</p></div><div><h3>Significance</h3><p>Understanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.</p></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"7 ","pages":"Pages 169-173"},"PeriodicalIF":2.0000,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9253402/pdf/","citationCount":"0","resultStr":"{\"title\":\"Myoclonus generators in sialidosis\",\"authors\":\"Felipe Vial , Patrick McGurrin , Sanaz Attaripour , Alesandra d'Azzo , Cynthia J. Tifft , Camilo Toro , Mark Hallett\",\"doi\":\"10.1016/j.cnp.2022.05.004\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objective</h3><p>Sialidosis 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.</p></div><div><h3>Methods</h3><p>Electromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1.</p></div><div><h3>Results</h3><p>The 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.</p></div><div><h3>Conclusion</h3><p>The 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.</p></div><div><h3>Significance</h3><p>Understanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.</p></div>\",\"PeriodicalId\":45697,\"journal\":{\"name\":\"Clinical Neurophysiology Practice\",\"volume\":\"7 \",\"pages\":\"Pages 169-173\"},\"PeriodicalIF\":2.0000,\"publicationDate\":\"2022-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9253402/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Neurophysiology Practice\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2467981X22000233\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"NEUROSCIENCES\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Neurophysiology Practice","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2467981X22000233","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"NEUROSCIENCES","Score":null,"Total":0}
Sialidosis 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.
Methods
Electromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1.
Results
The 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.
Conclusion
The 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.
Significance
Understanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.
期刊介绍：
Clinical Neurophysiology Practice (CNP) is a new Open Access journal that focuses on clinical practice issues in clinical neurophysiology including relevant new research, case reports or clinical series, normal values and didactic reviews. It is an official journal of the International Federation of Clinical Neurophysiology and complements Clinical Neurophysiology which focuses on innovative research in the specialty. It has a role in supporting established clinical practice, and an educational role for trainees, technicians and practitioners.
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