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https://www.readbyqxmd.com/read/30080186/lambert-eaton-myasthenic-syndrome-associated-with-extrapulmonary-small-cell-cancer-detected-by-18f-fdg-pet-ct
#1
Guoqian Chen, Zhanli Fu, Xueqi Chen, Qian Li
A 46-year-old woman suffering from lower extremity weakness, dysarthria, dysphagia, dyspnea, and dry mouth was suspected of having Lambert-Eaton myasthenic syndrome based on the characteristic electromyographic abnormalities of right ulnar nerve, which demonstrated reduced resting compound muscle action potential amplitude and greater than 100% incremental change in high-frequency repetitive nerve stimulation. An F-FDG PET/CT was performed to detect the underlying neoplasm and revealed a retroperitoneal soft tissue mass with high F-FDG avidity in the left iliac fossa...
September 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/30011414/pathogenic-mechanisms-and-clinical-correlations-in-autoimmune-myasthenic-syndromes
#2
Hakan Cetin, Angela Vincent
Autoimmune myasthenic syndromes are antibody-mediated disorders of the neuromuscular junction. Common antigenic targets are the acetylcholine receptor or muscle specific kinase (MuSK) in myasthenia gravis (MG) and the voltage-gated calcium channel in Lambert-Eaton myasthenic syndrome. There is evidence that antibodies directed against other antigens such as low-density lipoprotein receptor-related protein 4 (LRP4) are also involved in MG. The mechanisms by which various antibodies exert their pathogenic effect depend on the IgG subclass and also the epitope location on the antigens...
June 2018: Seminars in Neurology
https://www.readbyqxmd.com/read/29987589/low-specificity-of-voltage-gated-calcium-channel-antibodies-in-lambert-eaton-myasthenic-syndrome-a-call-for-caution
#3
Rodica Di Lorenzo, Karin Mente, Jianbo Li, Luay Shayya, Alexander Rae-Grant, Yuebing Li, Adham Jammoul
As testing for neuronal antibodies become more readily available, the spectrum of conditions potentially associated with these autoantibodies has been widening. Voltage-gated calcium channel antibodies (VGCC-Ab) are no exception to this trend. The significance of an elevated VGCC-Ab titer beyond its original clinicopathological correlate, Lambert-Eaton myasthenic syndrome (LEMS) remains undetermined. We sought to determine the diagnostic significance of an elevated serum VGCC-Ab titer in a large single-center cohort of 100 patients...
July 9, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29891101/-paraneoplastic-syndromes-in-ophthalmology-french-version-of-the-article
#4
REVIEW
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Although these syndromes are rare, the clinical manifestations can herald an unsuspected, underlying malignancy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Outlined in this review article are diagnostic features useful in differentiating cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic polymorphous vitelliform maculopathy, bilateral diffuse uveal melanocytic proliferation and paraneoplastic neurologic syndromes such as optic neuropathy, opsoclonus-myoclonus, Lambert-Eaton myasthenia and paraneoplastic cerebellar degeneration...
June 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29879051/coexistence-of-myasthenia-gravis-and-lambert-eaton-myasthenic-syndrome-in-a-small-cell-lung-cancer-patient-a-case-report
#5
Rui Jia, Jinbo Chen, Ruli Ge, Qi Zheng, Fang Chen, Zhonghua Zhao
RATIONALE: Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are both neuromuscular junction diseases, and some controversy exists whether the 2 diseases occur at the same time. PATIENT CONCERNS: We report a case that a patient with presentation of acetylcholine receptor (AChR) antibody positive MG and LEMS associated with small cell lung cancer (SCLC). DIAGNOSES: The patient firstly suffered from fluctuant symptoms, including slurred speech, double eyelid ptosis, and weakness of limbs...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29784239/paraneoplastic-syndromes-in-ophthalmology
#6
REVIEW
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Chorioretinal involvement is seen in CAR (cancer-associated retinopathy) syndrome, MAR (melanoma-associated retinopathy) syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation...
May 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29742721/coexistence-of-lambert-eaton-myasthenic-syndrome-and-autoimmune-encephalitis-with-anti-crmp5-cv2-and-anti-gabab-receptor-antibodies-in-small-cell-lung-cancer-a-case-report
#7
Hongfang Li, Aimei Zhang, Yanlei Hao, Hongzhi Guan, Zhanyun Lv
RATIONALE: Autoimmune encephalitis and Lambert-Eaton myasthenic syndrome are classic paraneoplastic neurological conditions common in patients with small cell lung cancer. PATIENT CONCERNS: The patient complained of tiredness, fluctuating recent memory loss, and inability to find his home. His family members reported a change in character, irritability, and paranoia. One month later, the patient had 1 grand mal seizure lasting 5 minutes. DIAGNOSIS: The patient was diagnosed with limbic encephalitis combined with Lambert-Eaton myasthenic syndrome...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29696584/how-to-spot-congenital-myasthenic-syndromes-resembling-the-lambert-eaton-myasthenic-syndrome-a-brief-review-of-clinical-electrophysiological-and-genetics-features
#8
REVIEW
Paulo José Lorenzoni, Rosana Herminia Scola, Claudia Suemi Kamoi Kay, Lineu Cesar Werneck, Rita Horvath, Hanns Lochmüller
Congenital myasthenic syndromes (CMS) are heterogeneous genetic diseases in which neuromuscular transmission is compromised. CMS resembling the Lambert-Eaton myasthenic syndrome (CMS-LEMS) are emerging as a rare group of distinct presynaptic CMS that share the same electrophysiological features. They have low compound muscular action potential amplitude that increment after brief exercise (facilitation) or high-frequency repetitive nerve stimulation. Although clinical signs similar to LEMS can be present, the main hallmark is the electrophysiological findings, which are identical to autoimmune LEMS...
June 2018: Neuromolecular Medicine
https://www.readbyqxmd.com/read/29681644/paraneoplastic-lambert-eaton-syndrome-in-a-patient-with-disseminated-metastatic-cancer
#9
Gregorio Arellano-Aguilar, Erik Santiago Núñez-Mojica, José Luis Gutiérrez-Velazco, Luis Gerardo Domínguez-Carrillo
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...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29655456/lambert-eaton-myasthenic-syndrome
#10
REVIEW
Vita G Kesner, Shin J Oh, Mazen M Dimachkie, Richard J Barohn
Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29526952/lambert-eaton-myasthenic-syndrome-associated-with-synchronous-double-cancer-a-combination-of-small-cell-carcinoma-of-the-cervix-and-breast-carcinoma
#11
Hiroyuki Fukuda, Akira Tanaka, Yasuyuki Hirashima, Ichiro Ito
Lambert-Eaton myasthenic syndrome (LEMS) is most commonly associated with small cell lung carcinoma, while it is rarely associated with gynecological and breast carcinoma. We herein report a case of LEMS associated with synchronous double cancer, which was a combination of small cell carcinoma of the cervix and breast carcinoma. The early diagnosis and treatment of LEMS are important for achieving a good outcome. The possibility of accompanying paraneoplastic neurological syndrome must be sufficiently considered in gynecology and breast cancer patients...
March 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29518096/myasthenia-gravis-seronegative-for-acetylcholine-receptor-antibodies-in-south-korea-autoantibody-profiles-and-clinical-features
#12
MULTICENTER STUDY
Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung-Joon Sung, Kwang-Kuk Kim, Young-Min Lim, Jee-Eun Kim, Byung-Jo Kim, Jin-Sung Park, Jeong-Geon Lim, Dae-Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung-Nam Yoon, Nam-Hee Kim, Suk-Won Ahn, Jeeyoung Oh, Hyung Jun Park, Kyong Jin Shin, Yoon-Ho Hong
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA)...
2018: PloS One
https://www.readbyqxmd.com/read/29507855/pure-ocular-weakness-as-the-initial-manifestation-of-lambert-eaton-myasthenic-syndrome
#13
Nakul Katyal, Raghav Govindarajan
Pure ocular presentation of Lambert-Eaton syndrome is not a common phenomenon. Such presentation poses significant diagnostic challenges and requires conscientious evaluation. In this review, we have described a case of a patient with pure ocular weakness, initially diagnosed as seronegative ocular myasthenia which on further evaluation was found to have ocular Lambert-Eaton myasthenic syndrome (LEMS).
December 31, 2017: Curēus
https://www.readbyqxmd.com/read/29489969/lambert-eaton-myasthenic-syndrome-the-60th-anniversary-of-eaton-and-lambert-s-pioneering-article
#14
Paulo José Lorenzoni, Cláudia Suemi Kamoi Kay, Lineu Cesar Werneck, Rosana Herminia Scola
This historical review describes the contribution of Drs. Lee M. Eaton and Edward H. Lambert to the diagnosis of myasthenic syndrome on the 60th anniversary of their pioneering article (JAMA 1957) on the disease. There are important landmarks in their article on a disorder of the neuromuscular junction associated with thoracic neoplasm and the electrophysiological criteria for Lambert-Eaton myasthenic syndrome (LEMS). After 60 years, the main electrophysiological criteria described in Eaton and Lambert's pioneering article are still currently useful in the diagnosis of LEMS...
February 2018: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/29402568/3-4-diaminopyridine-for-the-treatment-of-myasthenia-gravis-with-electrophysiological-patterns-of-lambert-eaton-myasthenic-syndrome
#15
Moon Kyu Lee, Il Nam Sunwoo, Seung Min Kim
3,4-Diaminopyridine (34DAP) is a presynaptic transmission enhancer. Its efficacy for Lambert-Eaton myasthenic syndrome (LEMS) and myasthenia gravis (MG) was demonstrated. However, there are cases sharing the characteristics of both disease and the effect of 34DAP in "gray zone" patients is sparse. Recently, we prescribed 34DAP to five anti-acetylcholine receptor antibody-positive MG patients with electrophysiological LEMS patterns and three LEMS patients, and carefully monitored the responses. Sero-positive MG patients exhibited more favorable responses than LEMS patients...
April 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29386498/-a-retrospective-study-of-the-effects-of-3-4-diaminopyridine-treatment-in-lambert-eaton-myasthenic-syndrome
#16
Ryoji Naganuma, Ichiro Yabe, Ikuko Takahashi, Masaaki Matsushima, Takahiro Kano, Hidenao Sasaki
In this independent clinical study, we analyzed retrospectively the clinical features of 9 cases (6 male and 3 female) of Lambert-Eaton myasthenic syndrome that were administered 3,4-diaminopyridine (3,4-DAP). Four cases showed no cancer and 5 cases had small cell lung carcinoma. Seven cases were positive for anti voltage-gated calcium channel antibodies. Activities of daily living (ADL) were improved by 3,4-DAP in 8 cases that showed mainly weakness of the extremities, but did not improve ADL in 1 case with cerebellar ataxia of paraneoplastic cerebellar degeneration (PCD)...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29377152/a-presynaptic-congenital-myasthenic-syndrome-attributed-to-a-homozygous-sequence-variant-in-lama5
#17
REVIEW
Ricardo A Maselli, Juan Arredondo, Jessica Vázquez, Jessica X Chong, Michael J Bamshad, Deborah A Nickerson, Marian Lara, Fiona Ng, Victoria Lee Lo, Peter Pytel, Craig M McDonald
We report a severe defect of neuromuscular transmission in a consanguineous patient with a homozygous variant in the laminin α5 subunit gene (LAMA5). The variant c.8046C > T (p.Arg2659Trp) is rare and has a predicted deleterious effect. The affected individual, who also carries a rare homozygous sequence variant in LAMA1, had normal cognitive function, but magnetic resonance brain imaging showed mild volume loss and periventricular T2 prolongation. Repetitive nerve stimulation at 2 Hz showed 50% decrement of compound muscle action potential amplitudes but 250% facilitation immediately after exercise, similar to that seen in Lambert-Eaton myasthenic syndrome...
February 2018: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29365352/lambert-eaton-myasthenic-syndrome-and-cerebellar-ataxia-is-response-to-immunotherapy-a-clue-to-pathogenesis
#18
EDITORIAL
Philip Alexander Ambrose, Paul Maddison
No abstract text is available yet for this article.
January 24, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29355452/retrospective-study-of-paraneoplastic-neurological-syndromes-in-a-chinese-han-population-from-shandong-east-china
#19
Shuai Miao, Shaohua Liao, Heng Li, Bing Niu, Huaiqiang Hu, Ying Qian, Hongwei Guo, Bingzhen Cao
OBJECTIVE: To analyze the clinical features, diagnostic strategies and therapeutic methods associated with paraneoplastic neurological syndromes. METHODS: A retrospective study of paraneoplastic neurological syndromes was performed at a single center in Shandong, East China. The medical records and follow-up data of 28 patients were intensively reviewed between February 2011 and December 2014. RESULTS: Twenty-four (85.7%) patients experienced subacute or chronic onset of disease, and the most common symptoms reported were mild myasthenia and paresthesias...
September 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29333908/false-positive-acetylcholine-receptor-antibodies-in-a-case-of-unilateral-chronic-progressive-external-ophthalmoplegia-case-report-and-review-of-literature
#20
Rehan Rajput, Amun Sachdev, Nizar Din, Erika Marie Damato, Aidan Murray
METHODS: We present a rare case with atypical presenting features of unilateral CPEO with a false positive Acetylcholine Receptor Antibody (AchRA) test resulting in diagnostic delay. We illustrate the unilateral nature of this case and demonstrate the caveats of performing myogenic ptosis correction in such patients. We also discuss the differential diagnosis of false positive AchRA, a test commonly performed in the investigation of ptosis. RESULTS: A 34-year old female presented with a more than 3-year history of slowly-progressive, unilateral, right-sided restriction in eye movements and ptosis...
January 15, 2018: Orbit
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