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J Int Med Res 2021 May;49(5):3000605211014784

Unit of Otorhinolaryngology, University Luigi Vanvitelli, Napoli, Italy.

This current report describes a rare clinical case of neuroendocrine carcinoma (NEC) of the larynx with associated Lambert-Eaton myasthenic paraneoplastic syndrome (LEMS). A 68-year-old male patient presented with severe dysphonia and dysphagia. He underwent a total laryngectomy and the excised lesion was extremely large. Read More

Cureus 2021 Mar 14;13(3):e13889. Epub 2021 Mar 14.

Clinical Neurophysiology Department, La Princesa University Hospital, Madrid, ESP.

Myasthenia gravis is a neuromuscular disease that causes weakness in skeletal muscles because of the presence of acetylcholine receptor antibodies. These antibodies produce a compromise in the end-plate potential, reducing the safety factor for effective synaptic transmission. Clinically, this manifests as muscle weakness and, in severe cases, respiratory failure. Read More

Acta Derm Venereol 2021 May 10;101(5):adv00452. Epub 2021 May 10.

Department of Medical Sciences and Human Oncology, Section of Dermatology, University of Turin, Via Cherasco 23, IT-12124 Torino, Italy. E-mail:

Can J Neurol Sci 2021 Apr 13:1-9. Epub 2021 Apr 13.

Neurology 2021 Mar 8. Epub 2021 Mar 8.

Int J Mol Sci 2021 Feb 28;22(5). Epub 2021 Feb 28.

Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.

Signal transduction at the neuromuscular junction (NMJ) is affected in many human diseases, including congenital myasthenic syndromes (CMS), myasthenia gravis, Lambert-Eaton myasthenic syndrome, Isaacs' syndrome, Schwartz-Jampel syndrome, Fukuyama-type congenital muscular dystrophy, amyotrophic lateral sclerosis, and sarcopenia. The NMJ is a prototypic cholinergic synapse between the motor neuron and the skeletal muscle. Synaptogenesis of the NMJ has been extensively studied, which has also been extrapolated to further understand synapse formation in the central nervous system. Read More

J Clin Neuromuscul Dis 2021 Mar;22(3):164-168

Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA.

Abstract: Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular junction disorder, and dermatomyositis (DM) is an idiopathic inflammatory myopathy. LEMS and DM are uncommon conditions that can present similarly and are often associated with autoantibodies. Concomitant LEMS and DM have only been reported a few times, and most of those cases were paraneoplastic. Read More

J Biol Chem 2021 Jan 16:100302. Epub 2021 Jan 16.

Department of Neuroscience, Center for Neuroscience, University of Pittsburgh, Pittsburgh, PA 15260, USA. Electronic address:

3,4-diaminopyridine (3,4-DAP) increases transmitter release from neuromuscular junctions (NMJs), and low doses of 3,4-DAP (estimated to reach ∼1 μM in serum) are the FDA-approved treatment for neuromuscular weakness caused by Lambert-Eaton Myasthenic Syndrome (LEMS). Canonically, 3,4-DAP is thought to block voltage-gated potassium (Kv) channels, resulting in prolongation of the presynaptic action potential (AP). However, recent reports have shown that low millimolar concentrations of 3,4-DAP have an off-target agonist effect on the Cav1 subtype ("L-type") of voltage-gated calcium (Cav) channels, and have speculated that this agonist effect might contribute to 3,4-DAP effects on transmitter release at the NMJ. Read More

Front Mol Neurosci 2020 3;13:610964. Epub 2020 Dec 3.

Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.

The neuromuscular junction (NMJ) is a highly specialized synapse between a motor neuron nerve terminal and its muscle fiber that are responsible for converting electrical impulses generated by the motor neuron into electrical activity in the muscle fibers. On arrival of the motor nerve action potential, calcium enters the presynaptic terminal, which leads to the release of the neurotransmitter acetylcholine (ACh). ACh crosses the synaptic gap and binds to ACh receptors (AChRs) tightly clustered on the surface of the muscle fiber; this leads to the endplate potential which initiates the muscle action potential that results in muscle contraction. Read More

Intern Med 2020 Dec 15. Epub 2020 Dec 15.

Department of Neurology, Kansai Electric Power Hospital, Japan.

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. Read More

Neurol Res Pract 2020 9;2:52. Epub 2020 Dec 9.

Department of Neurology, Medical Faculty of the RWTH Aachen University, Aachen, Germany.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare, autoimmune or paraneoplastic condition characterized by muscle weakness and fatigability. In cancer therapy, immune checkpoint inhibitors (ICI) sensitize the immune system for tumor antigens. We report a 62-year-old, female patient with paraneoplastic LEMS as first manifestation of Merkel cell carcinoma. Read More

J Int Med Res 2020 Dec;48(12):300060520974466

Department of Neurology and Neuroscience Center, First Hospital of Jilin University, Changchun, China.

Patients with anti-CV2/collapsin response mediator protein (CRMP)5 antibodies present with more frequent chorea, cerebellar ataxia, uveo/retinal symptoms, and Lambert-Eaton myasthenic syndrome or myasthenia gravis. Chronic intestinal pseudo-obstruction (CIPO) is an intestinal motility dysfunction disease dysmotility that is caused by a neuromuscular disease with recurrent or persistent intestinal obstruction in the absence of mechanical obstruction. We report the case of a patient with CRMP5 antibody-positive paraneoplastic neurological syndrome (PNS) that is associated with autonomic dysfunction (presenting most remarkably as CIPO). Read More

Muscle Nerve 2021 03 20;63(3):E14-E15. Epub 2020 Dec 20.

Department of Neurology, St. John's Medical College Hospital, Bengaluru, India.

Muscle Nerve 2021 03 29;63(3):E18-E21. Epub 2020 Dec 29.

Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, 19104, Pennsylvania.

Muscle Nerve 2021 03 21;63(3):E16-E18. Epub 2020 Dec 21.

Disability Studies of Peripheral Nerve and Muscle, Graduate School of Health Sciences, Sapporo Medical University, Sapporo, Japan.

Rinsho Shinkeigaku 2020 Dec 20;60(12):852-856. Epub 2020 Nov 20.

Department of Neurology, Akita Red Cross Hospital.

A 77-year-old man with a history of lung cancer at the age of 71 developed involuntary right leg movement for a month. Neurological examination revealed a right-sided hemi-chorea. Autoimmune disease was suspected owing to the presence of oligoclonal bands and the elevated IgG-index in the cerebrospinal fluid. Read More

Neurol Clin 2021 02 7;39(1):133-146. Epub 2020 Nov 7.

Neurology Department, Indiana University School of Medicine, Indiana University Health, Indianapolis, Indiana, USA.

Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are the most common disorders of neuromuscular transmission in clinical practice. Disorders of the neuromuscular junction (NMJ) are characterized by fluctuating and fatigable weakness and include autoimmune, toxic, and genetic conditions. Adults with NMJ disorders are most often antibody mediated, with MG being the most common, having a prevalence of approximately 1 in 10,000, and with women being affected about twice as often as men. Read More

Drugs Today (Barc) 2020 Oct;56(10):623-641

Distinguished Professor of Neurology Emeritus, Department of Neurology, University of Alabama at Birmingham, Alabama, USA.

Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic autoimmune disabling neuromuscular disease caused by antibodies against presynaptic voltage-gated calcium channels. It reduces the quantal release of acetylcholine (Ach), causing muscle weakness, reduced or absent reflex and dysautonomia. About half of LEMS patients have associated small cell lung cancer. Read More

Neurodegener Dis Manag 2020 12 14;10(6):409-423. Epub 2020 Oct 14.

Department of Medicine, San Filippo Neri Hospital, Neurology Unit, Rome, Italy.

Symptomatic treatment has a great relevance for the management of patients with neurologic diseases, since it reduces disease burden and improves quality of life. Aminopyridines (APs) are a group of potassium (K+) channel blocking agents that exert their activity both at central nervous system level and on neuromuscular junction. This review describes the use of APs for the symptomatic treatment of neurological conditions. Read More

J Clin Neurol 2020 Oct;16(4):530-546

Geriatric Neurological Department of the Second Medical Centre, National Clinical Research Center of Geriatric Diseases, Chinese PLA General Hospital, Beijing, China.

Anti-Sry-like high mobility group box (SOX) 1 antibodies (abs) are partly characterized onconeural autoantibodies (autoabs) due to their correlation with neoplastic diseases. Anti-SOX1 abs are associated with various clinical manifestations, including Lambert-Eaton myasthenic syndrome (LEMS) and paraneoplastic cerebellar degeneration (PCD). However, the clinical characteristics of patients with anti-SOX1 abs have not been described in detail. Read More

Ther Adv Neurol Disord 2020 25;13:1756286420949808. Epub 2020 Aug 25.

Department of Neurology, St. Josef Hospital Bochum, Ruhr University of Bochum, Gudrunstrasse 56, Bochum, 44791, Germany.

Neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AE), myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are antibody-mediated neurological diseases. They have mostly female predominance, affecting many women during childbearing age. Interactions between the underlying disease (or necessary treatment) and pregnancy can occur in every of these illnesses. Read More

Muscle Nerve 2020 12 29;62(6):742-745. Epub 2020 Sep 29.

Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, USA.

Introduction: When performing postexercise facilitation (PEF) as part of the repetitive nerve stimulation (RNS) test in Lambert-Eaton myasthenic syndrome (LEMS), it is important to avoid any influence of the previous exercise or RNS test on the compound muscle action potential (CMAP) amplitude.

Methods: To measure the CMAP amplitude return time (ART) to that at rest, a single CMAP was obtained every 30 seconds until the amplitude was within 5% of that at rest in three exercise periods (10, 20, and 30 seconds) and in 10-second postexercise (PE) 3-Hz RNS testing with 17 tests in 10 LEMS patients.

Results: Adequate ART between tests is 150 seconds for 10-second exercise (Ex) and 10-second PE 3-Hz RNS test, 120 seconds for 20-second Ex, and 90 seconds for 30-second Ex. Read More

Clin Neuropathol 2021 Mar-Apr;40(2):93-97

Aims: Lambert-Eaton myasthenic syndrome (LEMS) is a kind of autoimmune disease of the neuromuscular junction that is often misdiagnosed as a peripheral nerve disease or myopathy. For some difficult cases, muscle biopsy examination is useful for differential diagnosis. However, studies about the pathological findings of LEMS patients are rare, especially of patients who were diagnosed with small cell lung carcinoma (SCLC). Read More

Front Oncol 2020 16;10:1074. Epub 2020 Jul 16.

Department of Thoracic Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, United States.

Small-cell lung cancer (SCLC) accounts for 13-15% of all new lung cancer cases in the US. The tumor has a tendency to disseminate early resulting in 80-85% of patients being diagnosed with extensive disease (ES-SCLC). Chemotherapy has provided SCLC patients considerable survival benefits over the past three decades. Read More

J Neurol Neurosurg Psychiatry 2020 10 10;91(10):1124-1125. Epub 2020 Jul 10.

Department of Clinical Neurosciences, Division of Neurology, Unit of Neuroimmunology and Multiple Sclerosis, University Hospitals Geneva, Geneva, Switzerland.

Pract Neurol 2020 Dec 6;20(6):435-445. Epub 2020 Jul 6.

Neuroscience and Trauma, Blizard Institute of Cell and Molecular Science, London, UK.

Vaccination is one of the most effective and cost-efficient methods for protecting people with multiple sclerosis (MS) from infections. However, use of vaccines has often been problematic because of misguided concerns that they may exacerbate the disease and/or that some disease-modifying therapies may influence the immune response to immunisations and/or their safety. People with MS risk higher morbidity and mortality from vaccine-preventable infections. Read More

Sci Rep 2020 06 29;10(1):10546. Epub 2020 Jun 29.

Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.

To evaluate the Dutch-English Lambert-Eaton Myasthenic Syndrome (LEMS) Tumour Association Prediction (DELTA-P) score in a prospective cohort of patients with newly diagnosed LEMS to assess the clinical validity of this tool in a real-world setting. Clinical features from 87 patients with LEMS, occurring within three months from disease onset, were collated to produce a DELTA-P score for each patient. Lung cancer was detected in 44/87 (51%) LEMS patients. Read More