{"doi":"10.1016/j.cnp.2022.05.004","title":"Myoclonus generators in sialidosis","abstract":"Objective: 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.","journal":"Clinical Neurophysiology Practice","year":2022,"id":276650,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":8,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9596,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2022-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":506894,"name":"Patrick Mcgurrin","orcid":"0000-0002-6962-9582","position":1,"is_corresponding":false},{"id":523451,"name":"Sanaz Attaripour","orcid":"0000-0002-4021-0307","position":2,"is_corresponding":false},{"id":947654,"name":"Alesandra d'Azzo","orcid":null,"position":3,"is_corresponding":false},{"id":70653,"name":"Cynthia J. Tifft","orcid":"0000-0002-3931-1207","position":4,"is_corresponding":false},{"id":260096,"name":"Camilo Toro","orcid":"0000-0002-3917-9031","position":5,"is_corresponding":false},{"id":255823,"name":"Mark Hallett","orcid":"0000-0002-3180-6811","position":6,"is_corresponding":false},{"id":347469,"name":"Felipe Vial","orcid":"0000-0002-3293-2924","position":0,"is_corresponding":true}],"reference_count":17,"raw_metadata":null,"created_at":"2026-07-19T00:28:30.709588Z","pmid":"35800887","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}