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Autonomic dysfunction in Lambert-Eaton myasthenic syndrome
R K Khurana1, C L Koski, R F Mayer
1Department of Neurology, University of Maryland School of Medicine, Baltimore 21201.
Journal of the Neurological Sciences
|May 1, 1988
Summary
Lambert-Eaton myasthenic syndrome (LEMS) can involve autonomic dysfunction, affecting both sympathetic and parasympathetic functions. Chemotherapy for small cell lung cancer (SCLC) in LEMS patients showed simultaneous improvement in autonomic symptoms and LEMS.
Area of Science:
- Neurology
- Autonomic Neuroscience
- Oncology
Background:
- Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder affecting neuromuscular transmission.
- Autonomic dysfunction is increasingly recognized as a potential manifestation of LEMS.
- Previous literature primarily identified cholinergic dysautonomia in LEMS.
Purpose of the Study:
- To investigate the spectrum of autonomic dysfunction in patients with Lambert-Eaton myasthenic syndrome.
- To explore the relationship between autonomic dysfunction, small cell lung cancer (SCLC), and LEMS.
- To assess the impact of SCLC treatment on autonomic function and LEMS severity.
Main Methods:
- Clinical assessment of autonomic symptoms in LEMS patients.
- Electrophysiological studies to document neuromuscular transmission defects.
- Laboratory investigations to evaluate sympathetic and parasympathetic function.
- Case study of a patient with SCLC-associated LEMS undergoing chemotherapy.
Main Results:
- Six patients with LEMS exhibited autonomic symptoms, indicating broader autonomic involvement than previously reported.
- Both sympathetic and parasympathetic nervous system abnormalities were identified.
- Four of the six patients had co-existing small cell lung cancer (SCLC).
- One patient experienced concurrent improvement in autonomic dysfunction and LEMS following SCLC chemotherapy, suggesting disease regression.
Conclusions:
- Autonomic dysfunction is a significant feature in a subset of LEMS patients, involving both sympathetic and parasympathetic systems.
- The co-occurrence of SCLC and LEMS may indicate a specific patient subgroup.
- Treatment of SCLC can lead to simultaneous improvement in both the cancer and associated LEMS, including autonomic dysfunction, hinting at a potential for better prognosis in these patients.