{"title":"非副肿瘤性兰伯特-伊顿肌无力综合征后的晚发性小脑共济失调综合征:一个案例研究。","authors":"David P Randall, Matthew C Randall","doi":"10.1186/s12883-024-03983-8","DOIUrl":null,"url":null,"abstract":"<p><p>This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy. He was diagnosed with LEMS through history, exam, electromyography/nerve conduction studies (EMG/NCS) with repetitive nerve stimulation (RNS) and antibody testing. He was treated with 3,4 diaminopyridine (3,4 DAP) with an excellent response. Five years later, he developed acute ataxia. The patient required months of intensive and continued immunomodulating therapy.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":"25 1","pages":"2"},"PeriodicalIF":2.2000,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11694385/pdf/","citationCount":"0","resultStr":"{\"title\":\"Late onset cerebellar ataxia syndrome after non-paraneoplastic Lambert-Eaton myasthenic syndrome: a case study.\",\"authors\":\"David P Randall, Matthew C Randall\",\"doi\":\"10.1186/s12883-024-03983-8\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy. He was diagnosed with LEMS through history, exam, electromyography/nerve conduction studies (EMG/NCS) with repetitive nerve stimulation (RNS) and antibody testing. He was treated with 3,4 diaminopyridine (3,4 DAP) with an excellent response. Five years later, he developed acute ataxia. The patient required months of intensive and continued immunomodulating therapy.</p>\",\"PeriodicalId\":9170,\"journal\":{\"name\":\"BMC Neurology\",\"volume\":\"25 1\",\"pages\":\"2\"},\"PeriodicalIF\":2.2000,\"publicationDate\":\"2025-01-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11694385/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"BMC Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1186/s12883-024-03983-8\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"BMC Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s12883-024-03983-8","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Late onset cerebellar ataxia syndrome after non-paraneoplastic Lambert-Eaton myasthenic syndrome: a case study.
This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy. He was diagnosed with LEMS through history, exam, electromyography/nerve conduction studies (EMG/NCS) with repetitive nerve stimulation (RNS) and antibody testing. He was treated with 3,4 diaminopyridine (3,4 DAP) with an excellent response. Five years later, he developed acute ataxia. The patient required months of intensive and continued immunomodulating therapy.
期刊介绍:
BMC Neurology is an open access, peer-reviewed journal that considers articles on all aspects of the prevention, diagnosis and management of neurological disorders, as well as related molecular genetics, pathophysiology, and epidemiology.