Abstract

The Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease of the neuromuscular transmission involving the presynaptic voltage-gated calcium channels (VGCCs). In most cases (60%), LEMS presents a paraneoplastic syndrome associated with small cell lung cancer, in the remaining an idiopathic, autoimmune diseases, with frequent overlap with other immune dysfunctions, including myasthenia gravis. The typical clinical triad in LEMS consists of weakness (predominantly affecting proximal muscles), autonomic dysfunctions (including xerostomia, pupillomotor dysfunction, constipation, impaired sweating) and hypo/areflexia. LEMS patients typically present a proximal lower > upper limb weakness that progressively involves axial, oculomotor and pharyngo-laryngeal muscles, thus leading to gait and upright alteration, ocular and bulbar symptoms in severely affected patients. We report the case of a 41-year-old woman diagnosed and treated from 2015. Due to the unusual initial symptoms and coexisting diseases, the patient remains under constant control and observation, including oncological monitoring.

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