{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T11:18:43Z","timestamp":1783423123852,"version":"3.54.6"},"reference-count":25,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T00:00:00Z","timestamp":1783382400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background and Clinical Significance: Lambert\u2013Eaton myasthenic syndrome (LEMS) is mediated by antibodies against P\/Q-type voltage-gated calcium channels (VGCCs) and is classified as paraneoplastic (T-LEMS) or non-paraneoplastic (NT-LEMS). Cerebellar degeneration is recognized in T-LEMS, but pathological confirmation in NT-LEMS has not been reported. Case Presentation: A 79-year-old man developed progressive ataxic gait and dysarthria at age 76 and was diagnosed with LEMS based on repetitive nerve stimulation findings and anti-P\/Q-type VGCC antibodies. No malignancy was identified during more than 40 months of surveillance, and comprehensive autopsy revealed no occult tumor. After hospitalization for erythroderma and pneumonia, he died of respiratory failure. Postmortem examination revealed severe Purkinje cell loss with Bergmann gliosis in the anterior lobe and tuber vermis, accompanied by torpedoes and empty baskets, without significant inflammation. These findings indicate that NT-LEMS can reach the same VGCC-associated Purkinje cell endpoint previously documented only in paraneoplastic LEMS, despite different upstream triggers. Conclusions: This first autopsy-confirmed case of NT-LEMS with cerebellar degeneration supports a shared, non-inflammatory VGCC-mediated pathway of Purkinje cell injury across LEMS subtypes.<\/jats:p>","DOI":"10.3390\/diagnostics16132124","type":"journal-article","created":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T09:53:46Z","timestamp":1783418026000},"page":"2124","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Autopsy-Confirmed Non-Paraneoplastic Lambert\u2013Eaton Myasthenic Syndrome with Cerebellar Degeneration: A Case Report"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2600-7549","authenticated-orcid":false,"given":"Hajime","family":"Iwata","sequence":"first","affiliation":[{"name":"Department of Pathology, Tokyo Metropolitan Tama Medical Center, Fuchu 183-8524, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2424-2218","authenticated-orcid":false,"given":"Jun","family":"Ikezawa","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu 183-0042, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masayuki","family":"Honda","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu 183-0042, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0774-2827","authenticated-orcid":false,"given":"Ryo","family":"Morishima","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu 183-0042, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuta","family":"Amagasaki","sequence":"additional","affiliation":[{"name":"Department of Emergency and General Medicine, Tokyo Metropolitan Tama Medical Center, Fuchu 183-8524, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-6154-5562","authenticated-orcid":false,"given":"Tomonari","family":"Seki","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Teishin Hospital, Chiyoda-ku, Tokyo 102-8798, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiro","family":"Kiriu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tokyo Metropolitan Tama Medical Center, Fuchu 183-8524, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Ishizawa","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine (Neuropathology), Tokyo Metropolitan Neurological Hospital, Fuchu 183-0042, Japan"},{"name":"Department of Pathology, Saitama Medical University, Saitama 350-0495, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazushi","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu 183-0042, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-7328-9034","authenticated-orcid":false,"given":"Haruka","family":"Okada","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tokyo Metropolitan Tama Medical Center, Fuchu 183-8524, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1016\/S1474-4422(11)70245-9","article-title":"Lambert-Eaton Myasthenic Syndrome: From Clinical Characteristics to Therapeutic Strategies","volume":"10","author":"Titulaer","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1016\/S1474-4422(21)00297-0","article-title":"Epidemiology, Diagnostics, and Biomarkers of Autoimmune Neuromuscular Junction Disorders","volume":"21","author":"Punga","year":"2022","journal-title":"Lancet Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1854","DOI":"10.1007\/s00415-017-8541-9","article-title":"Lambert-Eaton Myasthenic Syndrome (LEMS): A Rare Autoimmune Presynaptic Disorder Often Associated with Cancer","volume":"264","author":"Schoser","year":"2017","journal-title":"J. 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