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Lambert Eaton Myasthenic Syndrome LEMS

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

Aili Wang, Xin Zhang, Jiawen Yi, Min Zhu, Yuhui Zhang,

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. In this case, L858R mutation was ... Read more >>

Thorac Cancer (Thoracic cancer)
[2020, :]

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Guidance for the management of myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) during the COVID-19 pandemic.

S. Jacob, S. Muppidi, A. Guidon, J. Guptill, M. Hehir, J.F. Howard, I. Illa, R. Mantegazza, H. Murai, K. Utsugisawa, J. Vissing, H. Wiendl, R.J. Nowak,

J. Neurol. Sci. (Journal of the neurological sciences)
[0, :]

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

Paul Maddison, Paul Gozzard, Girija Sadalage, Philip A Ambrose, Caroline J Chapman, Andrea Murray, Selina Thomsen, Antonio Berretta, Bethan Lang,

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 ... Read more >>

J. Neuroimmunol. (Journal of neuroimmunology)
[2020, 340:577149]

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

Chunyang Li, Xiaolei Wang, Lihua Sun, Hui Deng, Yanqiu Han, Wenqi Zheng,

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 ... Read more >>

Thorac Cancer (Thoracic cancer)
[2020, 11(2):465-469]

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

Alexander F Lipka, Marion I Boldingh, Erik W van Zwet, Marco W J Schreurs, Jan B M Kuks, Chantal M Tallaksen, Maarten J Titulaer, Jan J G M Verschuuren,

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 ... Read more >>

Neurology (Neurology)
[2020, 94(5):e511-e520]

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

Amanda C Guidon,

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 ... Read more >>

Continuum (Minneap Minn) (Continuum (Minneapolis, Minn.))
[2019, 25(6):1785-1806]

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

Renato Mantegazza,

Introduction: 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.Areas ... Read more >>

Expert Rev Clin Pharmacol (Expert review of clinical pharmacology)
[2019, 12(11):1013-1018]

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Amifampridine for the treatment of Lambert-Eaton myasthenic syndrome.

Shin J Oh,

Introduction: The present status of amifampridine (AFP) for the treatment of Lambert-Eaton myasthenic syndrome (LEMS) is reviewed. Areas covered: All relevant literature identified through a PubMed search under treatment of LEMS, aminopyridine, and amifampridine are reviewed. An expert opinion on AFP was formulated. Expert opinion: AFPs, 3,4-DAP and 3,4-DAPP, are ... Read more >>

Expert Rev Clin Immunol (Expert review of clinical immunology)
[2019, 15(10):991-1007]

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Thymic small cell carcinoma associated with Lambert-Eaton myasthenic syndrome.

Saeko Hashimoto, Kazuki Hayasaka, Katsuyuki Suzuki, Makoto Endoh, Naoki Yanagawa, Satoshi Shiono,

Thymic small cell cancer is a very rare type of thymic epithelial tumor. Lambert-Eaton myasthenic syndrome (LEMS) is a rare paraneoplastic syndrome associated with thymic epithelial tumors. We report an extremely rare case of LEMS associated with thymic small cell carcinoma. A 71-year-old man was referred to our institution for ... Read more >>

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2019, :]

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Coexistence of Anti-SOX1 and Anti-GABAB Receptor Antibodies with Autoimmune Encephalitis in Small Cell Lung Cancer: A Case Report.

Wei Qin, Xiao Wang, Jing Yang, Wenli Hu,

Anti-γ-aminobutyric acid B receptor (anti-GABABR) encephalitis is a rare type of autoimmune encephalitis (AE). Although it responds well to immunomodulating therapy and has favorable prognosis, anti-GABABR AE has often been misdiagnosed as infectious encephalitis. Herein, we present a case of a 59-year-old female with anti-GABABR AE associated with small cell ... Read more >>

Clin Interv Aging (Clinical interventions in aging)
[2020, 15:171-175]

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Associations between HLA and autoimmune neurological diseases with autoantibodies.

Sergio Muñiz-Castrillo, Alberto Vogrig, Jérôme Honnorat,

Recently, several autoimmune neurological diseases have been defined by the presence of autoantibodies against different antigens of the nervous system. These autoantibodies have been demonstrated to be specific and useful biomarkers, and most of them are also pathogenic. These aspects have increased the value of autoantibodies in neurological practice, as ... Read more >>

Auto Immun Highlights (Autoimmunity Highlights)
[2020, 11(1):2]

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

Arsalan Anwar, Sidra Saleem, Mirza Fawad Ahmed, Sara Ashraf, Sameen Ashraf,

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 ... Read more >>

Cureus (Cureus)
[2019, 11(8):e5450]

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

Natsuko Ishida, Yuya Kondo, Yuri Chikano, Erina Kobayashi-Nakade, Yukio Suga, Junko Ishizaki, Kiyonobu Komai, Ryo Matsushita,

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 ... Read more >>

Biopharm Drug Dispos (Biopharmaceutics & drug disposition)
[2019, 40(8):294-301]

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

Fumitaka Shimizu, Yukio Takeshita, Yasuteru Sano, Yuka Hamamoto, Hirokazu Shiraishi, Takuya Sato, Shunsuke Yoshimura, Toshihiko Maeda, Susumu Fujikawa, Hideaki Nishihara, Hiroko Kitanosono, Akira Tsujino, Masakatsu Motomura, Takashi Kanda,

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. ... Read more >>

Brain (Brain : a journal of neurology)
[2019, 142(8):2253-2264]

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

Connie H Yoon, Jocelyn Owusu-Guha, Adam Smith, Pamela Buschur,

Objective: 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. Data Sources: PubMed, ... Read more >>

Ann Pharmacother (The Annals of pharmacotherapy)
[2020, 54(1):56-63]

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

Shruti M Raja, Donald B Sanders, Vern C Juel, Yadollah Harati, A Gordon Smith, Robert Pascuzzi, David P Richman, Angie Wu, Kathy L Aleš, Laura R Jacobus, David P Jacobus, Jeffrey T Guptill, ,

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 ... Read more >>

Muscle Nerve (Muscle & nerve)
[2019, 60(3):292-298]

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

Kavita Agrawal, Nirav Agrawal,

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 ... Read more >>

Case Rep Neurol Med (Case reports in neurological medicine)
[2019, 2019:5353202]

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

Yiqi Liu, Jianying Xi, Lei Zhou, Hui Wu, Dongyue Yue, Wenhua Zhu, Jie Lin, Jiahong Lu, Chongbo Zhao, Kai Qiao,

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 ... Read more >>

J Clin Neurosci (Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia)
[2019, 65:41-45]

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

Oriol Mirallas, Nuria Rial, Berta Martín-Cullell, Jesus Recio-Iglesias,

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 ... Read more >>

BMJ Case Rep (BMJ case reports)
[2019, 12(7):]

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Neuronal Antibodies and Associated Syndromes.

Borros M Arneth,

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 >>

Autoimmune Dis (Autoimmune diseases)
[2019, 2019:2135423]

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Congenital myasthenic syndrome with mild intellectual disability caused by a recurrent SLC25A1 variant.

Sunitha Balaraju, Ana Töpf, Grace McMacken, Veeramani Preethish Kumar, Astrid Pechmann, Helen Roper, Seena Vengalil, Kiran Polavarapu, Saraswati Nashi, Niranjan Prakash Mahajan, Ines A Barbosa, Charu Deshpande, Robert W Taylor, Judith Cossins, David Beeson, Steven Laurie, Janbernd Kirschner, Rita Horvath, Robert McFarland, Atchayaram Nalini, Hanns Lochmüller,

Congenital myasthenic syndromes (CMS) are a clinically and genetically heterogeneous group of disorders caused by mutations which lead to impaired neuromuscular transmission. SLC25A1 encodes a mitochondrial citrate carrier, associated mainly with the severe neurometabolic disease combined D-2- and L-2-hydroxyglutaric aciduria (D/L-2-HGA). We previously reported a single family with a homozygous ... Read more >>

Eur. J. Hum. Genet. (European journal of human genetics : EJHG)
[2020, 28(3):373-377]

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Lambert-Eaton myasthenic syndrome and merkel cell carcinoma.

Nam D Nguyen, Daniel B Simmons, Adrian R Bersabe, Thomas M Duginski, John H Sladky, Douglas Walton, Micah Will, John S Renshaw,

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 ... Read more >>

Cutis (Cutis)
[2019, 103(5):E19-E23]

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Autoimmune Channelopathies at Neuromuscular Junction.

Kun Huang, Yue-Bei Luo, Huan Yang,

The neuromuscular junction, also called myoneural junction, is a site of chemical communication between a nerve fiber and a muscle cell. There are many types of channels at neuromuscular junction that play indispensable roles in neuromuscular signal transmission, such as voltage-gated calcium channels and voltage-gated potassium channels on presynaptic membrane, ... Read more >>

Front Neurol (Frontiers in Neurology)
[2019, 10:516]

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

Hiroko Kitanosono, Masakatsu Motomura, Hiroki Tomita, Hiroshi Iwanaga, Naoki Iwanaga, Takashi Irioka, Hirokazu Shiraishi, Akira Tsujino,

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 ... Read more >>

Brain Nerve (Brain and nerve = Shinkei kenkyu no shinpo)
[2019, 71(2):167-174]

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Symptomatic treatment of botulism with a clinically approved small molecule.

Edwin Vazquez-Cintron, James Machamer, Celinia Ondeck, Kathleen Pagarigan, Brittany Winner, Paige Bodner, Kyle Kelly, M Ross Pennington, Patrick McNutt,

Botulinum neurotoxins (BoNTs) are potent neuroparalytic toxins that cause mortality through respiratory paralysis. The approved medical countermeasure for BoNT poisoning is infusion of antitoxin immunoglobulins. However, antitoxins have poor therapeutic efficacy in symptomatic patients; thus, there is an urgent need for treatments that reduce the need for artificial ventilation. We ... Read more >>

JCI Insight (JCI insight)
[2020, 5(2):]

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