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Mohammad Abu-Tineh, Mohammed A Alamin, Esra'a Aljaloudi, Awni Alshurafa, Beatriz Garcia-Cañibano, Ruba Y Taha, Sarah A Elkourashy
INTRODUCTION: Lambert-Eaton myasthenia syndrome (LEMS) is a rare autoimmune disorder characterized by autoantibodies targeting presynaptic neuromuscular junctions. It results in muscle weakness and autonomic dysfunction. LEMS can be idiopathic or associated with neoplastic diseases, often small-cell lung cancer. This case report describes a rare instance of paraneoplastic LEMS in a man with non-Hodgkin lymphoma. CASE PRESENTATION: A 57-year-old male with non-Hodgkin lymphoma presented with progressive muscle weakness, diminished reflexes, and autonomic symptoms...
2023: Case Reports in Oncology
#22
Athanasios Zamparas, Torsten Herzog, Sarah Förster, Ines Siglienti, Waldemar Uhl, Johanna Strotmann, Philipp Höhn
BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of the neuromuscular junction. It can occur as a paraneoplastic disorder associated with various types of carcinomas, usually small cell lung cancer or as an autoimmune disease. LEMS can be misdiagnosed as myasthenia gravis or as an oncological sequela, causing delays in diagnosis. We present a rare case of a male adult with confirmed LEMS occurring with pancreatic carcinoma. CASE DESCRIPTION: A 66-year-old man presented with a newly diagnosed pancreatic tumor...
2023: AME Case Reports
#23
Yasuharu Oda, Hironobu Tsubouchi, Nobuyuki Ishii, Aya Kitamura, Eiji Moriyama, Eriko Mitsutome, Katsuya Sakai, Kazutaka Shiomi, Shigehisa Yanagi, Taiga Miyazaki
Small cell lung carcinoma (SCLC) is a neuroendocrine carcinoma with a poor prognosis and is a common cause of paraneoplastic syndromes. Paraneoplastic syndromes are characterized by neurological and endocrinological problems in patients with malignancy and are often associated with difficulty in induction of chemotherapy. Here we report the case of a patient with SCLC concomitant with two paraneoplastic syndromes, syndrome of inappropriate antidiuretic hormone secretion (SIADH) and Lambert-Eaton myasthenic syndrome (LEMS), who was treated with a platinum-doublet chemotherapy regimen...
2023: Respiratory Medicine Case Reports
#24
JOURNAL ARTICLE
Brynhildur Thors, Bjarni Gudmundsson
Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neurological disorder caused by autoimmune antibodies attacking the presynaptic neuromuscular junction, in some cases caused by underlying cancer. The main clinical finding is fluctuating weakness of the extremities and a triad of symtoms can help physicians suspect the disease. A key to the diagnosis are the electrophysiological abnormalities seen in this group of diseases. Treatment with symtomatic and/or immunosuppressive therapy is important as well as a workup for possible malignancy...
November 2023: Læknablađiđ
#25
REVIEW
Mario Pace, Roberto Cannella, Vincenzo Di Stefano, Antonino Lupica, Paolo Alonge, Giulio Morici, Filippo Brighina, Federica Brancato, Federico Midiri, Massimo Galia
Autoimmune neuromuscular diseases are a group of heterogenous pathologies secondary to the activation of the immune system that damage the structures of the peripheric nerve, the neuromuscular junction, or the skeleton muscle. The diagnosis of autoimmune neuromuscular disorders comprises a combination of data from clinical, laboratory, electromyography, imaging exam, and biopsy. Particularly, the whole-body MRI examination in the last two decades has been of great use in the assessment of neuromuscular disorders...
October 23, 2023: Brain Sciences
#26
JOURNAL ARTICLE
Hao Liu, Yumin Li, Jie Li, Qiongchi Zhang, Jingtao Wu, Xinyu Li, Liesu Meng, Shuai Cao, Haopeng Li
Background: The musculoskeletal toxicity of immune checkpoint inhibitors (ICIs) is receiving increasing attention with clinical experience. Nevertheless, the absence of a systematic investigation into the musculoskeletal toxicity profile of ICIs currently results in the under-recognition of associated adverse events. Further and more comprehensive investigations are warranted to delineate the musculoskeletal toxicity profile of ICIs and characterize these adverse events. Material and methods: The present study employed the FDA Adverse Event Reporting System database to collect adverse events between January 2010 and March 2021...
2023: Frontiers in Pharmacology
#27
JOURNAL ARTICLE
Syed Tabish Rehman, Syeda Mahnoor Azeem, Areeba Akbar, Omer Shafiq, Syed Shehryar Azeem, Shabbir Hussain A Ali
Nivolumab is an anti-programmed cell death protein 1 (anti-PD-1) monoclonal antibody and was the first immune checkpoint inhibitor drug approved for use in advanced non-small cell lung cancer (NSCLC). In this report, we describe a rare case of Lambert-Eaton myasthenic syndrome (LEMS), which developed as a side effect of nivolumab in a patient with metastatic lung squamous cell carcinoma. Our patient, who was previously treated with nivolumab for metastatic squamous cell carcinoma of the lung, appeared with a headache, swollen face, dysarthria, asthenia, xerostomia, and drooping eyelid...
October 14, 2023: Journal of Chemotherapy
#28
JOURNAL ARTICLE
Kazuki Yamada, Hiroaki Yaguchi, Kaede Ishikawa, Daiki Tanaka, Yuki Oshima, Keiichi Mizushima, Hisashi Uwatoko, Shinichi Shirai, Ikuko Iwata, Masaaki Matsushima, Keiko Tanaka, Ichiro Yabe
A 74-year-old man experienced diplopia, generalized muscle weakness, and acute respiratory failure. He was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) and treated with immunotherapy, but no improvement was observed, and additional symptoms, including central apnea and hallucinations, appeared. Subsequent serum and cerebrospinal fluid (CSF) analyses confirmed the presence of GABAB receptor antibodies, indicating the coexistence of autoimmune encephalitis. Although there were no findings of malignancy, it is highly likely that occult small-cell lung carcinoma was present...
September 22, 2023: Internal Medicine
#29
JOURNAL ARTICLE
Lewis G Halsey, Vincent Careau, Philip N Ainslie, Heliodoro Alemán-Mateo, Lene F Andersen, Liam J Anderson, Leonore Arab, Issad Baddou, Linda Bandini, Kweku Bedu-Addo, Ellen E Blaak, Stephane Blanc, Alberto G Bonomi, Carlijn V C Bouten, Pascal Bovet, Soren Brage, Maciej S Buchowski, Nancy Butte, Stephan G Camps, Regian Casper, Graeme L Close, Lisa H Colbert, Jamie A Cooper, Richard Cooper, Prasangi Dabare, Sai Krupa Das, Peter S W Davies, Sanjoy Deb, Christine Delisle Nyström, William Dietz, Lara R Dugas, Simon Eaton, Ulf Ekelund, Asmaa El Hamdouchi, Sonja Entringer, Terrence Forrester, Barry W Fudge, Melanie Gillingham, Annelies H Goris, Michael Gurven, Hinke Haisma, Catherine Hambly, Daniel Hoffman, Marije B Hoos, Sumei Hu, Noorjehan Joonas, Annemiek Joosen, Peter Katzmarzyk, Kitty P Kempen, Misaka Kimura, William E Kraus, Wantanee Kriengsinyos, Rebecca Kuriyan, Robert F Kushner, Estelle V Lambert, Pulani Lanerolle, Christel L Larsson, Nader Lessan, Marie Löf, Corby Martin, Eric Matsiko, Gerwin A Meijer, James C Morehen, James P Morton, Aviva Must, Marian Neuheuser, Theresa A Nicklas, Robert M Ojiambo, Kirsi H Pietilainen, Yannis P Pitsiladis, Jacob Plange-Rhule, Guy Plasqui, Ross L Prentice, Roberto Rabinovich, Susan B Racette, David A Raichen, Eric Ravussin, Leanne Redman, John J Reilly, Rebecca Reynolds, Susan Roberts, Jennifer C Rood, Dulani Samaranayake, Luís B Sardinha, Albertine J Scuitt, Analiza M Silva, Srishti Sinha, Anders M Sjödin, Eric Stice, Albert Stunkard, Samuel S Urlacher, Mauro Eduardo Valencia, Giulio Valenti, Ludo M van Etten, Edgar A Van Mil, Jeanine A Verbunt, Jonathan C K Wells, George Wilson, Brian Wood, Tsukasa Yoshida, Xueying Zhang, Alexia Murphy-Alford, Cornelia Loechl, Amy Luke, Herman Pontzer, Jennifer Rood, Hiroyuki Sagayama, Klaas R Westerterp, William W Wong, Yosuke Yamada, John R Speakman
There is considerably greater variation in metabolic rates between men than between women, in terms of basal, activity and total (daily) energy expenditure (EE). One possible explanation is that EE is associated with male sexual characteristics (which are known to vary more than other traits) such as musculature and athletic capacity. Such traits might be predicted to be most prominent during periods of adolescence and young adulthood, when sexual behaviour develops and peaks. We tested this hypothesis on a large dataset by comparing the amount of male variation and female variation in total EE, activity EE and basal EE, at different life stages, along with several morphological traits: height, fat free mass and fat mass...
September 2023: Biology Letters
#30
JOURNAL ARTICLE
Katherine M Clifford, Connie K Wu, David Post, Ruba Shaik, Srikanth Muppidi
OBJECTIVES: Sensitivity and specificity of Repetitive Nerve Stimulation (RNS) is typically reported from outpatient centers, and we hypothesized that these values might not apply to hospitalized patients with higher grades of weakness. RNS may be helpful in rapidly confirming diagnosis of myasthenia gravis (MG) in the inpatient setting, as results from confirmatory antibody testing are often delayed. We sought to characterize the sensitivity and specificity of RNS in the inpatient setting to assist in the early diagnosis of MG...
October 2023: Neurohospitalist
#31
JOURNAL ARTICLE
Jenny J Yoon, Alexandra L Anderson, Adam D Niesen
OBJECTIVE: In this case report, we discuss the rare manifestation of prolonged neuromuscular blockade in a patient with history of small cell lung cancer and undiagnosed Lambert-Eaton myasthenic syndrome (LEMS) who had previously received succinylcholine for general anesthesia without incident but subsequently exhibited prolonged neuromuscular blockade during a laparoscopic procedure. We aimed to emphasize the importance of reversal agent safety and precision as well as vigilant perioperative and postoperative care...
September 12, 2023: Clinical Neuropharmacology
#32
Michimi Otani, Miki Nishimori, Hitomi Iwasa, Mamiko Iwamura, Takayasu Izumi, Kosuke Nakaji, Noriko Nitta, Kana Miyatake, Rika Yoshimatsu, Tomoaki Yamanishi, Tomohiro Matsumoto, Yasushi Osaki, Noriko Wada, Makoto Toi, Marino Yamamoto, Yu Nakatani, Tetsuya Kubota, Takuji Yamagami
Spontaneous regression (SR) of cancer is very rare, especially of small cell lung cancer (SCLC). Recently, an association of paraneoplastic neurological syndrome (PNS) has been reported as a cause of SR of cancer, and onconeural antibodies are a possible factor in the SR of cancer associated with PNS. We herein report the first case of SR of SCLC combined with anti-P/Q-type of voltage-gated calcium channel (VGCC) antibody-positive Lambert-Eaton myasthenic syndrome (LEMS), a subtype of PNS. This case report suggests that SCLC may be spontaneously reduced by an autoimmune response induced by VGCC antibodies associated with LEMS...
November 2023: Radiology Case Reports
#33
JOURNAL ARTICLE
Chutithep Teekaput, Kanokkarn Teekaput, Surat Tanprawate, Adisak Kittisares, Metha Apiwattanakul
PURPOSE: The coexistence of IgLON5-IgG and SOX1-IgG is rare. Previous reports have shown that patients with IgLON5-IgG spectrum disease present with sleep disorders, bulbar involvement, and autonomic abnormality, while SOX1-IgG positive patients present with peripheral nervous system symptoms such as the Lambert-Eaton Myasthenic Syndrome (LEMS). CASE REPORT: We report a patient who presented with progressive ophthalmoplegia, ptosis, oropharyngeal dysphagia, gait instability, and sleep disorders...
September 30, 2023: Acta Neurologica Taiwanica
#34
JOURNAL ARTICLE
Michelle Fm Ten Brinck, Inge Wh Verheijen, Jaron van de Wardt, Gert W van Dijk, Frouke Ap Nijhuis, Aad Verrips
BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular junction disorder and the clinical triad consists of proximal muscle weakness, autonomic symptoms and reduced tendon reflexes. Sluggish pupillary reflexes are common but dilated fixed pupils are rare. CASE PRESENTATION: We report a patient with a rare clinical course of LEMS. The patient was hospitalised due to progressive dyspnoea. She was ambulant and independent of oxygen at hospitalisation...
2023: BMJ neurology open
#35
Silvia Falso, Gregorio Spagni, Raffaele Iorio, Amelia Evoli
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune neuromuscular junction disorder, paraneoplastic in 55% of cases and commonly associated with small-cell lung cancer (SCLC). We report the case of a 61-year-old man presented who with a 3-month history of lower limb proximal weakness, progressing to upper limbs, associated with dysphagia, xerostomia and erectile dysfunction. Electrodiagnostic studies and anti voltage-gated calcium channel (VGCC) antibodies (Abs) detection confirmed LEMS diagnosis. Contrast-enhanced thorax computed tomography (CT) scan and subsequently [18 F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) showed no malignancy...
September 2023: Neuromuscular Disorders: NMD
#36
REVIEW
Jinny Tavee, Thomas H Brannagan, Michael W Lenihan, Sri Muppidi, Liz Kellermeyer, Peter D Donofrio
Intravenous immune globulin (IVIG) is an immune-modulating biologic therapy that is increasingly being used in neuromuscular disorders despite the paucity of high-quality evidence for various specific diseases. To address this, the AANEM created the 2009 consensus statement to provide guidance on the use of IVIG in neuromuscular disorders. Since then, there have been several randomized controlled trials for IVIG, a new FDA-approved indication for dermatomyositis and a revised classification system for myositis, prompting the AANEM to convene an ad hoc panel to update the existing guidelines...
October 2023: Muscle & Nerve
#37
JOURNAL ARTICLE
Masakatsu Motomura, Takashi Irioka
Approximately 90% of patients with Lambert-Eaton myasthenic syndrome (LEMS) are positive for P/Q-type voltage-gated calcium channels (VGCCs) antibodies, and can be broadly divided into two groups: paraneoplastic, especially with small cell lung carcinoma and, non-paraneoplastic, without cancer. Under the Japanese LEMS diagnostic criteria 2022, abnormal electrophysiological results is mandatory for diagnosis in addition to muscle weakness. Contrastingly, autoantibodies are useful for diagnosing the etiology and influence treatment strategies...
July 2023: Brain and Nerve, Shinkei Kenkyū No Shinpo
#38
JOURNAL ARTICLE
Marion Gra, Anne Pham-Ledard, Emilie Gerard, Caroline Dutriaux, Marie Beylot-Barry, Fanny Duval, Louis Carla, Antoine Soulages, Sorilla Prey
Merkel cell carcinoma (MCC) is an aggressive neuroendocrine cutaneous tumor with high metastatic potential. In rare cases, it can be associated with paraneoplastic syndromes (PNS), which result from an antitumor immunity against antigens produced by the tumor itself. Lambert-Eaton Myasthenic Syndrome (LEMS) is a neurological autoimmune PNS characterized by an impairment of the neuromuscular junction, leading to proximal muscle weakness and fatigability. Although the development of immune checkpoint inhibitors (ICI) is a breakthrough in the management of many cancers, onset or worsen of immune diseases has been described...
June 19, 2023: Journal of Immunotherapy
#39
JOURNAL ARTICLE
Yeo-Dim Park, Yoon-Jee Chae, Han-Joo Maeng
Amifampridine is a drug used for the treatment of Lambert-Eaton myasthenic syndrome (LEMS) and was approved by the Food and Drug Administration (FDA) of the United States (US) in 2018. It is mainly metabolized by N -acetyltransferase 2 (NAT2); however, investigations of NAT2-mediated drug interactions with amifampridine have rarely been reported. In this study, we investigated the effects of acetaminophen, a NAT2 inhibitor, on the pharmacokinetics of amifampridine using in vitro and in vivo systems. Acetaminophen strongly inhibits the formation of 3- N -acetylamifmapridine from amifampridine in the rat liver S9 fraction in a mixed inhibitory manner...
May 11, 2023: Pharmaceutics
#40
JOURNAL ARTICLE
Tomohiro Uemura, Jun Sawada, Ikkei Ohashi, Bungo Hirose, Rika Yamauchi
The patient, a 58-year-old man, experienced weakness of the proximal muscles in both lower extremities, and Lambert-Eaton myasthenic syndrome and small cell carcinoma of unknown primary origin were diagnosed. He received symptomatic treatment for myasthenia and radiochemotherapy for small cell carcinoma; once this regimen, the myasthenic symptoms improved. However, acute myocardial infarction occurred, after which type II respiratory failure developed, and the patient required ventilator management with tracheal intubation...
June 28, 2023: Rinshō Shinkeigaku, Clinical Neurology
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