Autopsy-Confirmed Non-Paraneoplastic Lambert-Eaton Myasthenic Syndrome with Cerebellar Degeneration: A Case Report.

Background and Clinical Significance: Lambert-Eaton 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.