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Am J Phys Med Rehabil 2020 Apr 21. Epub 2020 Apr 21.

Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, U.S.A.

Muscle Nerve 2020 Apr 15. Epub 2020 Apr 15.

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

Background: Increment of compound muscle action potential amplitude is a diagnostic hallmark of Lambert-Eaton myasthenic syndrome (LEMS). Making a diagnosis can be challenging, therefore, a proper cutoff for abnormal increment is highly relevant for improved recognition of this rare disease.

Methods: We determined the sensitivity and specificity of 60% and 100% cutoff values in all consecutive patients who underwent increment testing in our hospital from 1999 to 2016. Read More

Am J Med Genet A 2020 Apr 6. Epub 2020 Apr 6.

Department of Anesthesiology, University of California Davis, Davis, California, USA.

Defects in the gene encoding synaptotagmin 2 (SYT2) have been linked to a presynaptic congenital myasthenic syndrome (CMS) and motor neuropathies. However, to date only dominant forms of the disease have been described. We report here a consanguineous patient with a severe recessive form of presynaptic CMS and denervation atrophy caused by the homozygous mutation c. Read More

J Neurol Sci 2020 05 25;412:116803. Epub 2020 Mar 25.

Department of Neurology, Yale University School of Medicine, New Haven, CT 06520, USA. Electronic address:

Neurology 2020 Apr 26;94(16):e1693-e1701. Epub 2020 Mar 26.

From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.

Objective: To validate the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test for diagnostic use in myasthenia gravis (MG) and to investigate its value in diagnostically challenging subgroups.

Methods: The RoVEMP test was performed in 92 patients with MG, 22 healthy controls, 33 patients with a neuromuscular disease other than MG (neuromuscular controls), 4 patients with Lambert-Eaton myasthenic syndrome, and 2 patients with congenital myasthenic syndrome.

Results: Mean decrement was significantly higher in patients with MG (28. Read More

Neuroscience 2020 Mar 17. Epub 2020 Mar 17.

Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, OX3 9DU, UK. Electronic address:

Myasthenia gravis (MG) is a relatively rare neurological disease that is usually associated with antibodies to the acetylcholine receptor (AChR). These antibodies (Abs) cause loss of the AChRs from the neuromuscular junction (NMJ), resulting in muscle weakness that can be life-threatening. Another form of the disease is caused by antibodies to muscle specific kinase (MuSK) that result in impaired AChR clustering and numbers at the NMJ, and may also interfere with presynaptic adaptive mechanisms. Read More

Thorac Cancer 2020 May 10;11(5):1334-1338. Epub 2020 Mar 10.

Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing Institute of Respiratory Medicine, Beijing, China.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare disease characterized by involvement of the neuromuscular junction. Most cases have an underlying malignancy, especially small-cell lung cancer (SCLC), while adenocarcinoma is less common. Here, we report a rare case of metastatic lung adenocarcinoma complicated with LEMS. Read More

J Neuroimmunol 2020 Mar 10;340:577149. Epub 2020 Jan 10.

Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford OX3 9DS, UK.

Since approximately 50% of patients with Lambert-Eaton myasthenic syndrome (LEMS) subsequently develop small-cell lung cancer (SCLC), it is important to be able to predict cancer occurrence in these patients at neurological presentation. We aimed to determine whether circulating biomarkers were effective and objective predictors of cancer development in LEMS. We found that the presence of either SOX2, N-type voltage gated calcium channel or GABAb antibodies at LEMS diagnosis was highly sensitive (84%) and specific (87%) for the detection of SCLC. Read More

Thorac Cancer 2020 Feb 27;11(2):465-469. Epub 2019 Dec 27.

Department of Clinical laboratory, Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China.

Paraneoplastic neurological syndromes (PNS) are rare disorders affecting any part of the central, peripheral or autonomic nervous system that occur in association with cancer. Among cancer patients, less than 1% overall develop PNS. Anti-SOX1 antibodies' positive paraneoplastic neurological disorders are rare and are usually associated with small cell lung cancer (SCLC). Read More

Neurology 2020 02 12;94(5):e511-e520. Epub 2019 Dec 12.

From the Departments of Neurology (A.F.L., J.J.G.M.V.) and Biostatistics (E.W.v.Z.), Leiden University Medical Center; Department of Neurology (A.F.L.), Groene Hart Hospital, Gouda, the Netherlands; Department of Neurology (M.I.B., C.M.T.), Oslo University Hospital, Norway; Departments of Immunology (M.W.J.S.) and Neurology (M.J.T.), Erasmus University Medical Center, Rotterdam; Department of Neurology (J.B.M.K.), University Medical Center Groningen, the Netherlands; and Faculty of Medicine (C.M.T.), University of Oslo, Norway.

Objective: To study survival and to characterize long-term functional impairments and health-related quality of life (HRQOL) of patients with Lambert-Eaton myasthenic syndrome (LEMS).

Methods: In this observational study, survival of patients with LEMS, separately for nontumor (NT) and small cell lung cancer (SCLC), was compared to that of the Dutch general population and patients with SCLC. Disease course in patients with LEMS was recorded retrospectively. Read More

Continuum (Minneap Minn) 2019 Dec;25(6):1785-1806

Purpose Of Review: This article reviews the pathophysiology, epidemiology, clinical presentation, diagnosis, and treatment of Lambert-Eaton myasthenic syndrome (LEMS) and of botulism, and immune-related myasthenia gravis (MG) occurring in the context of immune checkpoint inhibitor therapy for cancer.

Recent Findings: The suspicion that LEMS is rare but also likely underdiagnosed is supported by recent epidemiologic data. A validated, LEMS-specific scale now exists to assess and monitor disease, and symptomatic and immunomodulatory treatments are available. Read More

Med Clin (Barc) 2019 Nov 25. Epub 2019 Nov 25.

Servicio de Urgencias, Hospital Universitario La Paz, Madrid, España.

J Clin Neuromuscul Dis 2019 Dec;21(2):119-121

Department of Neurosciences and Mental Health, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.

J Clin Neuromuscul Dis 2019 Dec;21(2):103-106

Department of Neurology and Clinical Neurophysiology, Methodist University Hospital, University of Tennessee Health Science Center, Memphis, TN.

A case of triple-negative myasthenia gravis Lambert-Eaton overlap syndrome with negative Agrin and LRP-4 antibodies. Myasthenia gravis (MG) is an autoimmune disorder that shares similar features with Lambert-Eaton myasthenic syndrome. The combined clinical and electrophysiological findings of MG and Lambert-Eaton myasthenic syndrome have been reported, these cases represent the so-called "myasthenia gravis Lambert-Eaton overlap syndrome" (MLOS). Read More

Neurology 2019 11;93(21):939-944

From the Departments of Pathology (A.H.) and Neurology (R.J.N., B.R.), Yale School of Medicine (T.T.), New Haven, CT.

Neurol Sci 2020 May 18;41(5):1277-1279. Epub 2019 Nov 18.

Department of Medical Oncology, Cancer Center, Qilu Hospital of Shandong University, Jinan, Shandong, China.

Expert Rev Clin Pharmacol 2019 Nov 22;12(11):1013-1018. Epub 2019 Oct 22.

Unità Operativa Neuroimmunologia e Malattie Neuromuscolari, Fondazione IRCCS Istituto Neurologico "Carlo Besta", Milan, Italy.

: Lambert-Eaton myasthenic syndrome is an autoimmune disease of the neuromuscular junction characterized by a presynaptic defect of neuromuscular transmission resulting in muscle weakness and fatigability. Diagnostic features are specific neurophysiological alterations and autoantibody detection. The present review is focused on the use of Amifampridine Phosphate to treat LEMS patients. Read More

Cureus 2019 Aug 21;11(8):e5450. Epub 2019 Aug 21.

Internal Medicine, Dow Medical College and Civil Hospital Karachi, Karachi, PAK.

Lambert-Eaton Myasthenic Syndrome (LEMS) is an autoimmune-mediated neurological disorder that manifests as muscle fatigue, diminished tendon reflexes, with symptoms of cholinergic overactivity. It can be associated with certain neoplastic conditions, the most common being small cell lung carcinoma (SCLC). The basic pathophysiology involved is antibody-mediated targeting of voltage-gated calcium channels (VGCC), which decreases the release of acetylcholine in the synaptic junction. Read More

Ann Thorac Surg 2020 May 3;109(5):e347-e348. Epub 2019 Oct 3.

Department of Thoracic Surgery, Yamagata Prefectural Central Hospital, Yamagata, Japan. Electronic address:

Thymic small cell cancer is a very rare type of thymic epithelial tumor. Lambert-Eaton myasthenic syndrome is a rare paraneoplastic syndrome associated with thymic epithelial tumors. We report an extremely rare case of Lambert-Eaton myasthenic syndrome associated with thymic small cell carcinoma. Read More

Nervenarzt 2020 Feb;91(2):150-152

Medizinische Fakultät, Ludwig-Maximilians-Universität München, München, Deutschland.

Expert Rev Clin Immunol 2019 10 30;15(10):991-1007. Epub 2019 Sep 30.

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

: The present status of amifampridine (AFP) for the treatment of Lambert-Eaton myasthenic syndrome (LEMS) is reviewed. : All relevant literature identified through a PubMed search under treatment of LEMS, aminopyridine, and amifampridine are reviewed. An expert opinion on AFP was formulated. Read More

Neurology 2019 09;93(10):459-460

From the Department of Neurology (L.A.C., A.D.M., G.A.M., C.W.D.), Aberdeen Royal Infirmary; and Institute of Applied Health Sciences (A.D.M.), University of Aberdeen, Foresterhill, UK.

Muscle Nerve 2019 12 10;60(6):648-657. Epub 2019 Sep 10.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Although myopathies and neuromuscular junction disorders are typically distinct, their coexistence has been reported in several inherited and acquired conditions. Affected individuals have variable clinical phenotypes but typically display both a decrement on repetitive nerve stimulation and myopathic findings on muscle biopsy. Inherited causes include myopathies related to mutations in BIN1, DES, DNM2, GMPPB, MTM1, or PLEC and congenital myasthenic syndromes due to mutations in ALG2, ALG14, COL13A1, DOK7, DPAGT1, or GFPT1. Read More

Am J Nurs 2019 09;119(9):23

Diane S. Aschenbrenner is an assistant professor at Notre Dame of Maryland University in Baltimore. She also coordinates Drug Watch:

Biopharm Drug Dispos 2019 Sep;40(8):294-301

Faculty of Pharmacy, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kakuma-machi, Kanazawa, Ishikawa, 920-1192, Japan.

Lambert-Eaton myasthenic syndrome (LEMS) is characterized by muscle weakness, amyotrophy, easy fatigability, and depressed tendon reflexes. 3,4-Diaminopyridine (3,4-DAP) is the recommended therapy for the treatment of LEMS. However, estimations of 3,4-DAP pharmacokinetics in human and animals, such as rats, are rarely reported because 3,4-DAP is an orphan drug for the treatment of a very rare disease (LEMS). Read More

Autoimmune Dis 2019 9;2019:2135423. Epub 2019 Jul 9.

Institute of Laboratory Medicine and Pathobiochemistry, Molecular Diagnostics, University Hospital of the Universities of Giessen and Marburg UKGM, Justus Liebig University Giessen, Giessen, Germany.

Introduction: Multiple well-recognized conditions, such as Lambert-Eaton myasthenic syndrome (LEMS) and myasthenia gravis (MG), have been associated with neuronal antibodies.

Materials And Methods: A search was performed using Embase, PubMed, and CINAHL. An initial search of each database was conducted using keywords and terms related to the aim of the current review. Read More

Neurol Sci 2019 Dec 3;40(12):2609-2615. Epub 2019 Aug 3.

Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Shandong University, Jinan, 250012, China.

Objective: To study compound muscle action potential (CMAP) decrement by low-frequency repetitive nerve stimulation (RNS) in different hand muscles of amyotrophic lateral sclerosis (ALS) patients and the relationship with split hand phenomenon and clinical manifestation.

Methods: Clinical and decrement data of 51 ALS patients who had done RNS in different hand muscles were retrospectively reviewed from November 2016 to July 2017. Decrement data of 24 myasthenia gravis (MG) and 20 Lambert Eaton myasthenia syndrome (LEMS) patients was also reviewed to compare decrement pattern with hand muscles of ALS patients. Read More

Postgrad Med J 2019 Oct 31;95(1128):573-574. Epub 2019 Jul 31.

Department of Neurology, University of Vermont Medical Center, Burlington, Vermont, USA.

Case Rep Neurol Med 2019 2;2019:5353202. Epub 2019 Jul 2.

Department of Internal Medicine, Overlook Medical Center, Summit, NJ 07901, USA.

We present a case of a 59-year-old male with a confirmed diagnosis of small-cell lung cancer (SCLC). He had progressive disease even after four cycles of cisplatin and etoposide chemotherapy and 21 cycles of radiation. He was therefore started on immunotherapy with nivolumab every 2 weeks and ipilimumab every 6 weeks. Read More

Ann Pharmacother 2020 01 18;54(1):56-63. Epub 2019 Jul 18.

OhioHealth Riverside Methodist Hospital, Columbus, OH, USA.

The purpose of this article is to review the literature for both 3,4-diaminopyridine (3,4-DAP) and amifampridine for the treatment of Lambert-Eaton myasthenic syndrome (LEMS). Amifampridine (Firdapse) is the salt form of 3,4-DAP and was approved by the Food and Drug Administration for the treatment of LEMS. PubMed, TRIP database, and EMBASE searches were conducted without a back date (current to June 2019) utilizing the following search terms: , and . Read More

BMJ Case Rep 2019 Jul 16;12(7). Epub 2019 Jul 16.

Internal Medicine Department, Hospital Vall d'Hebron, Barcelona, Spain.

Paraneoplastic syndromes (PS) are a rare presentation of cancer, most commonly associated with small cell lung cancer (SCLC), breast cancer and haematologic malignancies. The diagnosis of PS is challenging because it could affect multiple organ systems and it may present before the tumour is visible by imaging. We report a malignant tumour diagnosed in a male patient who referred long-term paraesthesia and proximal muscle strength loss. Read More

Muscle Nerve 2019 09 18;60(3):292-298. Epub 2019 Jul 18.

Department of Neurology, Duke University Medical Center, Durham, North Carolina.

Introduction: There are no validated, practical, and quantitative measures of disease severity in Lambert-Eaton myasthenia (LEM).

Methods: Data from the Effectiveness of 3,4-Diaminopyridine in Lambert-Eaton Myasthenic Syndrome (DAPPER) trial were analyzed to assess triple timed up-and-go (3TUG) reproducibility and relationships between 3TUG times and other measures of LEM severity.

Results: The coverage probability technique showed ≥0. Read More

Neurohospitalist 2019 Jul 24;9(3):165-168. Epub 2018 Sep 24.

Neurology Department, Instituto de Investigaciones Neurológicas Raúl Carrea, FLENI, Buenos Aires, Argentina.

Anti-SOX1 antibodies are associated with diverse neurological syndromes, targeting both the central (paraneoplastic cerebellar degeneration) and peripheral nervous systems (Lambert Eaton myasthenic syndrome, paraneoplastic neuropathy). Although the pathogenic role of these antibodies remains unclear, their strong association with underlying neoplastic disease (mainly small-cell lung cancer) has designated them as onconeural antibodies. Here, we present a case of cerebellar ataxia with marked photophobia, with severe atrophy of the cerebellum and brain stem, associated with anti-SOX1 antibodies without evidence of an underlying malignancy. Read More

Brain 2019 08;142(8):2253-2264

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan.

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction caused by autoantibodies binding to P/Q-type voltage-gated calcium channels. Breakdown of the blood-brain barrier and diffusion of cerebellar granule/Purkinje cell-reactive autoantibodies into the CNS are critical for the pathogenesis of paraneoplastic cerebellar degeneration (PCD) with Lambert-Eaton myasthenic syndrome. We recently found evidence that glucose-regulated protein 78 (GRP78) autoantibodies in the plasma of patients with neuromyelitis optica promote the CNS access of AQP4 autoantibodies. Read More

Cutis 2019 May;103(5):E19-E23

Department of Hematology and Oncology, San Antonio Military Medical Center, Fort Sam Houston, Texas, USA.

Lambert-Eaton myasthenic syndrome (LEMS) is an antibody-mediated disorder of the neuromuscular junction that is most commonly diagnosed in association with small cell lung carcinoma (SCLC). Small cell lung carcinoma is histologically similar to the aggressive cutaneous neuroendocrine malignancy Merkel cell carcinoma (MCC). We provide a full report and longitudinal clinical follow-up of a case of LEMS occurring with MCC. Read More

Degener Neurol Neuromuscul Dis 2019 13;9:27-37. Epub 2019 May 13.

Neurology Department, Hospital Universitari Son Espases, Palma de Mallorca, Balearic Islands, Spain.

Lambert-Eaton myasthenic syndrome (LEMS) is an uncommon disorder of neuromuscular transmission with distinctive pathophysiological, clinical, electrophysiological and laboratory features. There are two forms of LEMS. The paraneoplastic (P-LEMS) form is associated with a malignant tumor that is most frequently a small cell lung carcinoma (SCLC), and the autoimmune (A-LEMS) form is often related to other dysimmune diseases. Read More

J Clin Neurosci 2019 Jul 6;65:41-45. Epub 2019 May 6.

Department of Neurology, Huashan Hospital, Fudan University, Shanghai 20040, China. Electronic address:

In order to describe the clinical characteristics, treatment response and long-term follow up in Lambert-Eaton myasthenic syndrome (LEMS) patients with and without small cell lung cancer (SCLC) in East China, patients seen in Huashan Hospital from January 1997 to December 2017 were included. Clinical information was collected retrospectively and quantitative MG (QMG) score, manual muscle testing (MMT), activities of daily living (ADL) scale were evaluated when the patients were followed up. Of 50 patients, 23 (46%) were SCLC-LEMS and 20 (40%) were nontumor LEMS (NT-LEMS). Read More

Cir Cir 2019 ;86(1):72-76

Rehabilitation Medicine, Faculty of Medicine, Universidad de Guanajuato. León, Gto., Mexico.

Background: Neurological paraneoplastic syndromes are rare, occur in 0.01% of all cancer patients; like part of them, the Lambert-Eaton syndrome is an autoimmune presynaptic disorder of neuromuscular transmission characterized by muscle weakness and neurovegetative dysfunction, and often associated with small cell lung cancer.

Case Report: A 72 years old female with a family history of lung cancer and leukemia, with 7 months of dry cough and 3 months with waist and pelvic muscle weakness, oropharyngeal dysphagia, dry mouth, chronic constipation and weight loss of 10 kg. Read More

J Clin Neuromuscul Dis 2019 Mar;20(3):111-119

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

Objective: To assess tolerability and efficacy of amifampridine phosphate versus placebo for symptomatic treatment of Lambert-Eaton Myasthenic Syndrome (LEMS).

Methods: This phase 3 randomized, double-blind, placebo-controlled withdrawal trial in 26 adults with LEMS compared efficacy of amifampridine phosphate versus placebo over a 4-day period. The primary endpoints were quantitative myasthenia gravis score (QMG) and subject global impression, and the secondary endpoint was Clinical Global Impression-Improvement. Read More

Brain Nerve 2019 Feb;71(2):167-174

Department of Neurology and Strokology, Nagasaki University Hospital.

A 63-year-old female who developed dizziness, diplopia and subsequent gait disturbance from September X-1 year was analyzed. The first neurological findings in May X year revealed cerebellar ataxia, weakness in the proximal limbs, decreased tendon reflexes, and autonomic symptoms (ADL:mRS 3). Furthermore, an incremental phenomenon was observed in the repetitive nerve stimulation test, and she was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) based on the serum P/Q-type calcium channel (VGCC) antibody positivity. Read More

Am J Ophthalmol 2019 04 14;200:242-249. Epub 2019 Jan 14.

Department of Ophthalmology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA. Electronic address:

Purpose: To report the incidence, demographics, and ocular findings of children with myasthenia.

Design: Retrospective cohort study.

Methods: The medical records of all children (<19 years) examined at Mayo Clinic with any form of myasthenia from January 1 1966, through December 31, 2015, were retrospectively reviewed. Read More

Case Rep Neurol 2018 Sep-Dec;10(3):346-352. Epub 2018 Dec 5.

Department of Respiratory Medicine, Osaka Police Hospital, Osaka, Japan.

Lambert-Eaton myasthenic syndrome (LEMS) is a representative paraneoplastic neurological syndrome. Recently, nivolumab, an anti-programmed cell death 1 inhibitor, has been approved for advanced non-small-cell lung cancer. Careful attention should be paid to immune-related adverse events (irAEs), including neurotoxicity. Read More

Radiology 2019 01;290(1):264-269

From the Department of Diagnostic Imaging, San Luigi Gonzaga Hospital, University of Torino, Regione Gonzole 10, Orbassano 10043 (Torino), Italy.

History A 29-year-old woman presented with a 6-month history of progressive general fatigue, fluctuating limb weakness, and difficulty climbing stairs. She initially experienced occasional episodes of transient diplopia that developed while reading in the evening. She subsequently started to experience dry eyes and mouth, difficulty chewing, and mild dysphagia that worsened throughout the day. Read More

Neurol Genet 2018 Dec 22;4(6):e282. Epub 2018 Oct 22.

Department of Neurology (N.I.M.-C., M.C., C.V., M.A.S.), University of Miami Miller School of Medicine FL; Department of Biology (Z.G., J.T.L.) and Department of Brain and Cognitive Sciences (Z.G., J.T.L.), The Picower Institute for Learning & Memory, Massachusetts Institute of Technology, Cambridge; and Department of Human Genetics (S.C., A.P.R., L.A., S.Z., M.A.S.), Hussman Institute for Human Genomics, University of Miami Miller School of Medicine, Miami, FL.

Objective: To report a new missense mutation causing distal hereditary motor neuropathy and presynaptic neuromuscular junction (NMJ) transmission dysfunction.

Methods: We report a multigenerational family with a new missense mutation, c. 1112T>A (p. Read More

Intern Med J 2018 12;48(12):1541-1542

Sunshine Hospital, Western Health, Melbourne, Victoria, Australia.

Muscle Nerve 2019 05 9;59(5):544-548. Epub 2019 Jan 9.

Department of Neurology, University of Alabama at Birmingham, UAB Station, Birmingham, Alabama, 35294, USA.

Introduction: Herein we report our experience with the repetitive nerve stimulation (RNS) test in myasthenia gravis (MG) crisis.

Methods: The various parameters of the RNS tests in 26 patients with MG crisis were analyzed.

Results: In 18 (69%) patients, MG crisis is the first manifestation of MG. Read More

J Neuroimmunol 2019 01 8;326:14-18. Epub 2018 Nov 8.

Department of Pathology, Nottingham University Hospitals NHS Trust, Queen's Medical Centre, Nottingham NG7 2UH, UK.

Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls. SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC. Read More

J Clin Neuromuscul Dis 2018 Dec;20(2):76-84

Pathology (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, PA.

This update begins with myasthenia gravis and the roles of anti-agrin and cortactin antibodies. Regarding diagnosis, a report on repeated ice pack testing is highlighted as are several reports on the close correlation of electrodiagnostic testing with clinical features and the response to treatment. The incidence of head drop and associated clinical and ventilatory features are gleaned from a retrospective study. Read More

Mult Scler Relat Disord 2019 Jan 22;27:131-132. Epub 2018 Oct 22.

Wake Forest School of Medicine, Department of Neurology, One Medical Center Blvd, Winston Salem, NC 27157, USA. Electronic address:

Background: Alemtuzumab administration is known to cause secondary autoimmune disease but has not been associated with the development of neurologic autoimmune conditions. Lambert-Eaton myasthenic syndrome (LEMS) is caused by autoantibodies directed against calcium channels on the neuromuscular junction.

Case Report: We report a case of a patient with relapsing-remitting multiple sclerosis (RRMS) treated with alemtuzumab who develop generalized weakness initially attributed to progression of MS but eventually determined to be due to LEMS. Read More