A patient with Lambert-Eaton myasthenic syndrome was clinically treated several times over a period of 9 months. During at least 3 weeks each time, the patient received prednisone, then underwent 9 plasmaphereses, and was given guanidine-hydrochloride (HCl) and 3,4-diaminopyridine (3,4-DAP). The muscle-power scoring, muscle function tests and the electrophysiological parameters showed a superiority of 3,4-DAP over the other pharmacological therapies. Good results were also obtained with plasmapheresis. Corticoid medication proved to be less effective. No improvement was seen with guanidine-HCl. For comparative judgement, two months after beginning the last therapy, the patient and the attending physician were questioned with regard to subjective therapeutic success. The answers largely match the objective results. Both favour 3,4-DAP. In contrast to the patient's opinion, the physician sees a distinct effect of plasmapheresis. All in all, the administration of 3,4-DAP may be seen as an effective and acceptable therapy in Lambert-Eaton myasthenic syndrome.
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BMC Neurol
January 2025
Neuromuscular Neurology, Advocate Health, 1850 Dempster Street, Park Ridge, IL, 60068, USA.
This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy.
View Article and Find Full Text PDFFront Neurol
December 2024
Department of Internal Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Background: Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of the presynaptic neuromuscular junction associated with antibody mediated dysfunction of voltage-gated calcium channels (VGCCs). LEMS can exist as a paraneoplastic syndrome, paraneoplastic-LEMS (P-LEMS), when associated with tumors, most commonly, small cell lung carcinoma (SCLC) or as a non-paraneoplastic condition (NP-LEMS) when no malignancies are detected.
Methods: A retrospective chart review was conducted in 3 tertiary hospitals in Saudi Arabia for patients diagnosed with LEMS between January 2010 and January 2020.
Acta Med Acad
August 2024
Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Background: Paraneoplastic Neurological Syndromes (PNS) constitute a heterogeneous cluster of disease manifestations related to various cancers. Small Cell Lung Cancer (SCLC) is strongly related to PNS. This narrative review conducted a survey in the available PubMed literature to highlight the appearance of PNSs in SCLC cases and discuss published research highlights on the subject so that general practitioners can be acquainted with the medical phenomenon present in SCLC patients.
View Article and Find Full Text PDFJ Clin Neuromuscul Dis
December 2024
Departments of Neurology and Pathology (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, PA.
This update starts with an interesting series of children and adults with congenital myasthenic syndrome with a DOK7 variant. The next section is on autoimmune myasthenia gravis (MG) epidemiology, cost of care, and hospitalizations. A number of studies on the newer treatments are discussed including a phase 2 trial of nipocalimab and recommendations for using some of these drugs.
View Article and Find Full Text PDFJ Neuroimmunol
January 2025
Department of Neurology, Mayo Clinic, Rochester, MN, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address:
Introduction: Paraneoplastic neurological syndromes (PNS) can worsen with immune checkpoint inhibitor (ICI) cancer immunotherapy.
Case Report: A 66-year-old female with paraneoplastic Lambert-Eaton Myasthenic Syndrome (LEMS), which led to the diagnosis of metastatic neuroendocrine carcinoma, was treated with intravenous immune globulin (IVIg) (with minimal response), chemotherapy, and radiation, resulting in neurological improvement. However, sclerodermatous changes developed after a year.
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