SynthesisNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026
Coexisting cerebellar ataxia and Lambert-Eaton myasthenic syndrome without malignancy: insights from a case and systematic literature review.
Synthesis in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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1 citing paper in PubMed.
- Autopsy-Confirmed Non-Paraneoplastic Lambert-Eaton Myasthenic Syndrome with Cerebellar Degeneration: A Case Report.Diagnostics (Basel, Switzerland) · 2026Article
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Abstract
backgroundLambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder often associated with small-cell lung carcinoma (SCLC). Cerebellar degeneration can coexist with LEMS, although their simultaneous presentation in the non-neoplastic setting is uncommon.
methodsWe describe a 75-year-old man with progressive cerebellar ataxia followed by bilateral ptosis, ophthalmoparesis, and proximal weakness. LEMS was confirmed by positive P/Q-type VGCC antibodies and electrophysiology. Extensive malignancy screening, including PET-CT, was negative, and immunotherapy produced marked neuromuscular and partial cerebellar improvement. To contextualize this case, we systematically reviewed the published literature (January 1991- August 2025) for this association, including cohorts, case reports and series providing individual patient-level details.
resultsWe identified 37 studies reporting 67 patients with coexistent LEMS and cerebellar ataxia. Malignancy was present in 71.6% (48/67), predominantly SCLC (35/67, 52.2%). The median interval from neurological symptom onset to cancer diagnosis was 3 months (IQR 0.5–6.0; range − 60 to 39 months). Anti-VGCC antibodies were detected in 83.6% (56/67), anti-SOX1 in 7.5% (5/67), and anti-Hu in 3.0% (2/67), while 6.0% (4/67) were seronegative. Notably, 28.4% (19/67) had no malignancy during follow-up, suggesting a non-paraneoplastic autoimmune subset. Neurological improvement was comparable between paraneoplastic and non-paraneoplastic cases, but mortality was significantly higher in malignancy-associated cases (27.1% vs. 5.3%, p = 0.04).
conclusionCoexistent LEMS and cerebellar ataxia is not invariably paraneoplastic. Early immunotherapy may improve neurological outcomes even in cancer-negative cases, but long-term oncologic surveillance remains essential.
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