1,123 results match your criteria Lambert-Eaton Myasthenic Syndrome


Use of Post-Exercise Facilitation to Assess Carpal Tunnel Syndrome with Concurrent Lambert-Eaton Myasthenic Syndrome.

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.

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http://dx.doi.org/10.1097/PHM.0000000000001448DOI Listing

Lowering the cutoff value for increment increases the sensitivity for the diagnosis of Lambert-Eaton myasthenic syndrome.

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

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http://dx.doi.org/10.1002/mus.26885DOI Listing

Recessive congenital myasthenic syndrome caused by a homozygous mutation in SYT2 altering a highly conserved C-terminal amino acid sequence.

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

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http://dx.doi.org/10.1002/ajmg.a.61579DOI Listing

Repetitive ocular vestibular evoked myogenic potentials in myasthenia gravis.

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

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http://dx.doi.org/10.1212/WNL.0000000000009306DOI Listing

ANTIBODIES AND RECEPTORS: From Neuromuscular Junction to Central Nervous System.

Authors:
Angela Vincent

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

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http://dx.doi.org/10.1016/j.neuroscience.2020.03.009DOI Listing

Successful treatment of advanced lung adenocarcinoma complicated with Lambert-Eaton myasthenic syndrome: A case report and literature review.

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

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http://dx.doi.org/10.1111/1759-7714.13385DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7180587PMC

Neuronal antibody detection and improved lung cancer prediction in Lambert-Eaton myasthenic syndrome.

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

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http://dx.doi.org/10.1016/j.jneuroim.2020.577149DOI Listing

Anti-SOX1 antibody-positive paraneoplastic neurological syndrome presenting with Lambert-Eaton myasthenic syndrome and small cell lung cancer: A case report.

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

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http://dx.doi.org/10.1111/1759-7714.13290DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6997017PMC
February 2020

Long-term follow-up, quality of life, and survival of patients with Lambert-Eaton myasthenic syndrome.

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

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http://dx.doi.org/10.1212/WNL.0000000000008747DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7080283PMC
February 2020

Lambert-Eaton Myasthenic Syndrome, Botulism, and Immune Checkpoint Inhibitor-Related Myasthenia Gravis.

Authors:
Amanda C Guidon

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

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http://dx.doi.org/10.1212/CON.0000000000000807DOI Listing
December 2019

Inadequate secretion of anti-diuretic hormone and Lambert-Eaton myasthenic syndrome as presentation of small cell lung cancer.

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

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

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http://dx.doi.org/10.1016/j.medcli.2019.09.018DOI Listing
November 2019

Lambert-Eaton Myasthenic Syndrome Plus.

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.

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http://dx.doi.org/10.1097/CND.0000000000000260DOI Listing
December 2019

A Case of Triple-Negative Myasthenia Gravis Lambert-Eaton Overlap Syndrome With Negative Agrin and LRP-4 Antibodies.

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

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http://dx.doi.org/10.1097/CND.0000000000000189DOI Listing
December 2019

Clinical Reasoning: Progressive proximal weakness in a 56-year-old man with bone pain.

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.

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http://dx.doi.org/10.1212/WNL.0000000000008535DOI Listing
November 2019

Non-small cell lung cancer associated with late-onset Lambert-Eaton myasthenic syndrome and paraneoplastic cerebellar degeneration.

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.

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http://dx.doi.org/10.1007/s10072-019-04139-0DOI Listing

Amifampridine tablets for the treatment of Lambert-Eaton myasthenic syndrome.

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

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http://dx.doi.org/10.1080/17512433.2019.1681972DOI Listing
November 2019

Recent Advances and Therapeutic Options in Lambert-Eaton Myasthenic Syndrome.

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

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http://dx.doi.org/10.7759/cureus.5450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6799875PMC
August 2019
2 Reads

Thymic Small Cell Carcinoma Associated With Lambert-Eaton Myasthenic Syndrome.

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

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http://dx.doi.org/10.1016/j.athoracsur.2019.08.080DOI Listing
May 2020
2 Reads

[Seronegative myasthenic syndrome?]

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

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

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http://dx.doi.org/10.1007/s00115-019-00810-1DOI Listing
February 2020

Amifampridine for the treatment of Lambert-Eaton myasthenic syndrome.

Authors:
Shin J Oh

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

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http://dx.doi.org/10.1080/1744666X.2020.1670061DOI Listing
October 2019
4 Reads

Reversible tongue atrophy in Lambert-Eaton myasthenic syndrome.

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.

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http://dx.doi.org/10.1212/WNL.0000000000008076DOI Listing
September 2019
2 Reads

Trouble at the junction: When myopathy and myasthenia overlap.

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

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http://dx.doi.org/10.1002/mus.26676DOI Listing
December 2019
5 Reads

First Drug Approved for Children with Rare Autoimmune Disorder.

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:

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http://dx.doi.org/10.1097/01.NAJ.0000580248.33784.ddDOI Listing
September 2019

Pharmacokinetics and tissue distribution of 3,4-diaminopyridine in rats.

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

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http://dx.doi.org/10.1002/bdd.2203DOI Listing
September 2019
2 Reads

Neuronal Antibodies and Associated Syndromes.

Authors:
Borros M Arneth

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

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http://dx.doi.org/10.1155/2019/2135423DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6652068PMC
July 2019
2 Reads

CMAP decrement by low-frequency repetitive nerve stimulation in different hand muscles of ALS patients.

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

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http://dx.doi.org/10.1007/s10072-019-04027-7DOI Listing
December 2019
1 Read
1.495 Impact Factor

Postexercise facilitation in Lambert-Eaton myasthenia syndrome.

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.

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http://dx.doi.org/10.1136/postgradmedj-2019-136843DOI Listing
October 2019

Lambert-Eaton Myasthenic Syndrome Secondary to Nivolumab and Ipilimumab in a Patient with Small-Cell Lung Cancer.

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

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http://dx.doi.org/10.1155/2019/5353202DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6633879PMC
July 2019
3 Reads

Amifampridine for the Management of Lambert-Eaton Myasthenic Syndrome: A New Take on an Old Drug.

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

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http://dx.doi.org/10.1177/1060028019864574DOI Listing
January 2020
4 Reads

A rare case of long-term paraesthesia diagnosed as a paraneoplastic syndrome by anti-SOX1 antibody determination.

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

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http://dx.doi.org/10.1136/bcr-2018-228916DOI Listing
July 2019
6 Reads

Validation of the triple timed up-and-go test in Lambert-Eaton myasthenia.

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

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http://dx.doi.org/10.1002/mus.26625DOI Listing
September 2019
3 Reads

Cerebellar Ataxia With Extreme Photophobia Associated With Anti-SOX1 Antibodies.

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

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http://dx.doi.org/10.1177/1941874418802130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6582382PMC
July 2019
8 Reads

GRP78 antibodies damage the blood-brain barrier and relate to cerebellar degeneration in Lambert-Eaton myasthenic syndrome.

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

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http://dx.doi.org/10.1093/brain/awz168DOI Listing
August 2019
6 Reads

Lambert-Eaton myasthenic syndrome and merkel cell carcinoma.

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

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May 2019
17 Reads

Lambert-Eaton Myasthenic syndrome: early diagnosis is key.

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

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http://dx.doi.org/10.2147/DNND.S192588DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6524763PMC
May 2019
8 Reads

Clinical characteristics and long term follow-up of Lambert-Eaton myasthenia syndrome in patients with and without small cell lung cancer.

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

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http://dx.doi.org/10.1016/j.jocn.2019.04.003DOI Listing
July 2019
10 Reads

Paraneoplastic Lambert-Eaton syndrome in a patient with disseminated metastatic cancer.

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

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http://dx.doi.org/10.24875/CIRUE.M18000011DOI Listing
December 2019
29 Reads

Amifampridine Phosphate (Firdapse) Is Effective in a Confirmatory Phase 3 Clinical Trial in LEMS.

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

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http://dx.doi.org/10.1097/CND.0000000000000239DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6392213PMC
March 2019
46 Reads

[Paraneoplastic Cerebellar Degeneration with Lambert-Eaton Myasthenic Syndrome: A Report of an Effectively Treated Case and Systematic Review of Japanese Cases].

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

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http://dx.doi.org/10.11477/mf.1416201237DOI Listing
February 2019
13 Reads

Incidence and Ocular Features of Pediatric Myasthenias.

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

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https://linkinghub.elsevier.com/retrieve/pii/S00029394193001
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http://dx.doi.org/10.1016/j.ajo.2019.01.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6587184PMC
April 2019
34 Reads

Lambert-Eaton Myasthenic Syndrome Caused by Nivolumab in a Patient with Squamous Cell Lung Cancer.

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

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https://www.karger.com/Article/FullText/494078
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http://dx.doi.org/10.1159/000494078DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323386PMC
December 2018
30 Reads

Case 261: Thymoma Embedded in Thymus with Pleural Implant in Myasthenia Gravis Lambert-Eaton Overlap Syndrome.

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

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http://dx.doi.org/10.1148/radiol.2018161761DOI Listing
January 2019
3 Reads

Identification of a new SYT2 variant validates an unusual distal motor neuropathy phenotype.

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

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http://dx.doi.org/10.1212/NXG.0000000000000282DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6244021PMC
December 2018
29 Reads

Lambert-Eaton myasthenic syndrome developing post-diagnosis of small-cell lung cancer.

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

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

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http://doi.wiley.com/10.1111/imj.14128
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http://dx.doi.org/10.1111/imj.14128DOI Listing
December 2018
44 Reads

Repetitive nerve stimulation test in myasthenic crisis.

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

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http://dx.doi.org/10.1002/mus.26390DOI Listing
May 2019
34 Reads

The utility of anti-SOX2 antibodies for cancer prediction in patients with paraneoplastic neurological disorders.

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

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http://dx.doi.org/10.1016/j.jneuroim.2018.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375907PMC
January 2019
17 Reads

What is in the Neuromuscular Junction Literature?

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

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http://dx.doi.org/10.1097/CND.0000000000000218DOI Listing
December 2018
11 Reads

Lambert-Eaton myasthenic syndrome associated with alemtuzumab administration.

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

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https://linkinghub.elsevier.com/retrieve/pii/S22110348183037
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http://dx.doi.org/10.1016/j.msard.2018.10.015DOI Listing
January 2019
19 Reads