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Journal articles on the topic 'LEMS'
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Sitcheran, Raquel, Roger Emter, Anastasia Kralli, and Keith R. Yamamoto. "A Genetic Analysis of Glucocorticoid Receptor Signaling: Identification and Characterization of Ligand-Effect Modulators in Saccharomyces cerevisiae." Genetics 156, no. 3 (November 1, 2000): 963–72. http://dx.doi.org/10.1093/genetics/156.3.963.
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Abstract To find novel components in the glucocorticoid signal transduction pathway, we performed a yeast genetic screen to identify ligand-effect modulators (LEMs), proteins that modulate the cellular response to hormone. We isolated several mutants that conferred increased glucocorticoid receptor (GR) activity in response to dexamethasone and analyzed two of them in detail. These studies identify two genes, LEM3 and LEM4, which correspond to YNL323w and ERG6, respectively. LEM3 is a putative transmembrane protein of unknown function, and ERG6 is a methyltransferase in the ergosterol biosynthetic pathway. Analysis of null mutants indicates that LEM3 and ERG6 act at different steps in the GR signal transduction pathway.
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Gilhus, Nils Erik. "Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy." Autoimmune Diseases 2011 (2011): 1–5. http://dx.doi.org/10.4061/2011/973808.
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Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disease with a well-characterized pathogenesis. In 50% of the patients, LEMS is a paraneoplastic manifestation and caused by a small cell lung carcinoma (SCLC). Both LEMS patients with SCLC and those without this tumour have in 85% of cases pathogenetic antibodies of very high LEMS specificity against voltage-gated calcium channels (VGCCs) in the cell membrane of the presynaptic motor nerve terminal. Better understanding of LEMS pathogenesis has lead to targeted symptomatic therapy aimed at the neuromuscular junction and to semispecific immuno-suppression. For SCLC LEMS, tumour therapy is essential.
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Lipka, Alexander F., Marion I. Boldingh, Erik W. van Zwet, Marco W. J. Schreurs, Jan B. M. Kuks, Chantal M. Tallaksen, Maarten J. Titulaer, and Jan J. G. M. Verschuuren. "Long‐term follow‐up, quality of life, and survival of patients with Lambert‐Eaton myasthenic syndrome." Neurology 94, no. 5 (December 12, 2019): e511-e520. http://dx.doi.org/10.1212/wnl.0000000000008747.
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ObjectiveTo study survival and to characterize long-term functional impairments and health-related quality of life (HRQOL) of patients with Lambert-Eaton myasthenic syndrome (LEMS).MethodsIn 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. Several scales for functional impairments and health-related quality of life were assessed.ResultsWe included 150 patients with LEMS. Survival was similar to that of the general population in 65 patients with NT-LEMS. Tumor survival was significantly longer in 81 patients with SCLC-LEMS compared to patients with non-LEMS SCLC (overall median survival 17 vs 7.0 months, p < 0.0001). At diagnosis, 39 (62%) of 63 patients with complete follow-up data were independent for activities of daily living, improving to 85% at the 1-year follow-up. The physical HRQOL composite score (55.9) was significantly lower than in the general population (76.3, p < 0.0001) and comparable to that of patients with myasthenia gravis (60.5). The mental HRQOL composite score was 71.8 in patients with LEMS, comparable to that of the general population (77.9, p = 0.19) and patients with myasthenia gravis (70.3).ConclusionsThis study shows that patients with NT-LEMS have normal survival. Patients with SCLC-LEMS have an improved tumor survival, even after correction for tumor stage. A majority of patients with LEMS report a stable disease course and remain or become independent for self-care after treatment.
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Mengelkamp, Esther, Julius Diesing, and Christof Weinhardt. "Tracing local energy markets: A literature review." it - Information Technology 61, no. 2-3 (April 24, 2019): 101–10. http://dx.doi.org/10.1515/itit-2019-0016.
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Abstract We conduct a structured literature review on the concept of local energy markets (LEMs). LEMs have gained increasing attention in the last two decades. Yet, a holistic definition and clear demarcation of LEMs is still missing. The review shows current works to shift their focus from conceptual implementation and design to increasingly realistic applications of LEMs. Secure access to (near) real-time smart meter data is a prerequisite for LEMs. Current research gaps, e. g. the inclusion of network constraints, agent-centric LEM designs or a comparison of market mechanisms, are identified.
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Sprekeler, Henning. "On the Relation of Slow Feature Analysis and Laplacian Eigenmaps." Neural Computation 23, no. 12 (December 2011): 3287–302. http://dx.doi.org/10.1162/neco_a_00214.
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The past decade has seen a rise of interest in Laplacian eigenmaps (LEMs) for nonlinear dimensionality reduction. LEMs have been used in spectral clustering, in semisupervised learning, and for providing efficient state representations for reinforcement learning. Here, we show that LEMs are closely related to slow feature analysis (SFA), a biologically inspired, unsupervised learning algorithm originally designed for learning invariant visual representations. We show that SFA can be interpreted as a function approximation of LEMs, where the topological neighborhoods required for LEMs are implicitly defined by the temporal structure of the data. Based on this relation, we propose a generalization of SFA to arbitrary neighborhood relations and demonstrate its applicability for spectral clustering. Finally, we review previous work with the goal of providing a unifying view on SFA and LEMs.
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Maddison, Paul, Paul Gozzard, Matthew J. Grainge, and Bethan Lang. "Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome." Neurology 88, no. 14 (March 1, 2017): 1334–39. http://dx.doi.org/10.1212/wnl.0000000000003794.
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Objective:To establish whether improved tumor survival in patients with Lambert-Eaton myasthenic syndrome (LEMS) and small-cell lung cancer (SCLC) was due to known prognostic risk factors or an effect of LEMS independently, perhaps as a result of circulating factors.Methods:We undertook a prospective observational cohort study of patients with LEMS attending Nottingham University Hospitals, UK, or via the British Neurological Surveillance Unit. In parallel, patients with a new diagnosis of biopsy-proven SCLC were enrolled, examined for neurologic illness, and followed up until death or study end.Results:Between May 2005 and November 2014, we recruited 31 patients with LEMS and SCLC and 279 patients with SCLC without neurologic illness. Allowing for known SCLC survival prognostic factors of disease extent, age, sex, performance status, and sodium values, multivariate Cox regression analysis showed that the presence of LEMS with SCLC conferred a significant survival advantage independently of the other prognostic variables (hazard ratio 1.756, 95% confidence interval 1.137–2.709, p = 0.011).Conclusions:Improved SCLC tumor survival seen in patients with LEMS and SCLC may not be due solely to lead time bias, given that survival advantage remains after allowing for other prognostic factors and that the same degree of survival advantage is not seen in patients with paraneoplastic neurologic syndromes other than LEMS presenting before SCLC diagnosis.
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Shimizu, Fumitaka, Yukio Takeshita, Yasuteru Sano, Yuka Hamamoto, Hirokazu Shiraishi, Takuya Sato, Shunsuke Yoshimura, et al. "GRP78 antibodies damage the blood–brain barrier and relate to cerebellar degeneration in Lambert-Eaton myasthenic syndrome." Brain 142, no. 8 (June 24, 2019): 2253–64. http://dx.doi.org/10.1093/brain/awz168.
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Abstract 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. In the present study, we investigated whether the GRP78 autoantibodies in PCD-LEMS IgG boost the brain uptake of cerebellar cell-reactive antibodies across the blood–brain barrier and facilitate cerebellar dysfunction. We first evaluated the effects of purified IgG from PCD-LEMS or PCD patients on the blood–brain barrier function in human brain microvascular endothelial cells using a high content imaging system with nuclear factor κB p65 and intracellular adhesion molecule 1 (ICAM1) immunostaining. Next, we identified GRP78 autoantibodies causing blood–brain barrier permeability in PCD-LEMS IgG by co-immunoprecipitation and the living cell-based antibody binding assays. Exposure of brain microvascular endothelial cells to IgG from PCD-LEMS patients induced nuclear factor κB p65 nuclear translocation, ICAM1 upregulation, reduced claudin-5 expression, increased permeability and increased autocrine IL-1β and IL-8 secretion; the IgG from patients with Lambert-Eaton myasthenic syndrome did not have these effects. We detected GRP78 autoantibodies in the IgG of LEMS-PCD (83.3%, n = 18), but observed fewer in patients with LEMS (6.6%, n = 15) and none were observed in the control subjects (n = 8). The depletion of GRP78 autoantibodies reduced the biological effect of LEMS-PCD IgG on brain microvascular endothelial cells. These findings suggest that GRP78 autoantibodies play a role beyond neuromyelitis optica and that they have direct implications in the phenotypic differences between PCD-LEMS and LEMS.
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Nakatani, Yuki, Natsuki Tanaka, Tomomi Enami, Seigo Minami, Tomoko Okazaki, and Kiyoshi Komuta. "Lambert-Eaton Myasthenic Syndrome Caused by Nivolumab in a Patient with Squamous Cell Lung Cancer." Case Reports in Neurology 10, no. 3 (December 5, 2018): 346–52. http://dx.doi.org/10.1159/000494078.
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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. We herein report a 73-year-old woman with LEMS that occurred during nivolumab treatment for pulmonary squamous cell carcinoma. After the 20th week of nivolumab, she experienced various neurological symptoms such as ptosis, lower limb weakness, and photophobia. Findings from a nerve conduction study and a positive anti-P/Q-type voltage-gated calcium channel antibody made a diagnosis of LEMS. Pyridostigmine and 3,4-diaminopyridine temporarily improved her symptoms. This was the first case of LEMS as a neurological irAE. LEMS should be considered as a possible neurological irAE.
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Li, Dan, Jiazhe Chen, An Wang, and Xiaoyun Wang. "Analysis of Software Implemented Low Entropy Masking Schemes." Security and Communication Networks 2018 (2018): 1–8. http://dx.doi.org/10.1155/2018/7206835.
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Low Entropy Masking Schemes (LEMS) are countermeasure techniques to mitigate the high performance overhead of masked hardware and software implementations of symmetric block ciphers by reducing the entropy of the mask sets. The security of LEMS depends on the choice of the mask sets. Previous research mainly focused on searching balanced mask sets for hardware implementations. In this paper, we find that those balanced mask sets may have vulnerabilities in terms of absolute difference when applied in software implemented LEMS. The experiments verify that such vulnerabilities certainly make the software LEMS implementations insecure. To fix the vulnerabilities, we present a selection criterion to choose the mask sets. When some feasible mask sets are already picked out by certain searching algorithms, our selection criterion could be a reference factor to help decide on a more secure one for software LEMS.
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Kohler, Siegfried, and Andreas Meisel. "The Lambert-Eaton Myasthenic Syndrome — an Overview." Neurology International Open 02, no. 01 (January 2018): E40—E45. http://dx.doi.org/10.1055/s-0043-118274.
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AbstractThe Lambert Eaton myasthenic syndrome (LEMS) has a prevalence of around 5/100 0000 and is around 10–20 times rarer than myasthenia gravis (MG). Although LEMS does have a number of similarities to MG, there are important differences. The syndrome is characterized by a mostly proximally localised exercise induced muscle weakness that can lead to respiratory failure often accompanied by autonomous dysfunction. Disease symptoms are caused by autoantibodies directed against P/Q type voltage gated calcium channels (VGCC) that are expressed in the presynaptic motoric nerve terminals. The diagnosis of LEMS is based on the detection of the pathogenic anti-VGCC antibodies as well as the observation of an increment of at least 60% in the electrophysiological examination of an affected muscle. An increment is defined by an increase of the at rest reduced compound muscle action potential (CMAP) either after voluntary maximal innervation or after high frequent (≥20 Hz) stimulation. In almost one third LEMS is of paraneoplastic origin. Therefore an intensive tumor screening is necessary after diagnosis.There are some differences in the clinical presentation between paraneoplastic (pLEMS) and the exclusively autoimmune (aiLEMS) form of LEMS. With respect to this the DELTA-P-Score and the detection of SOX1-antibody are important. The most frequent tumor associated with LEMS is small cell lung carcinoma (SCLC). Therapy is based on the initial distinction between paraneoplastic and autoimmune ethiology. pLEMS necessitates therapy of underlying neoplasia. Usually, aiLEMS- as well as pLEMS patients respond well to 3,4 diaminopyridine (3,4 DAP) often augmented by pyridostigmine. Similar to treatment of myasthenia gravis long-term immunosuppressive treatment is usually required to control symptoms effectively. Myasthenic crisis in LEMS can be controlled by intensive care and immunoglobulins, plasmaphereses or immunoadsorption. Based on case reports more specific immunomodulatory treatment approaches such as the B-cell depleting therapeutic antibody rituximab should be considered in therapy refractory courses of LEMS. Long-term prognosis of autoimmune LEMS with respect to clinical stabilization with (pharmacological) remission is good, although in around 75% of patients significant reductions in quality of life remain. Prognosis of tumor-associated LEMS is largely determined by the tumor and its effective therapy. Curative treatment of the tumour as well as complete remission of pLEMS are possible.
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Lorenzoni, Paulo J., Rosana H. Scola, Cláudia S. Kamoi Kay, Sérgio F. Parolin, and Lineu C. Werneck. "Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases." Arquivos de Neuro-Psiquiatria 68, no. 6 (December 2010): 849–54. http://dx.doi.org/10.1590/s0004-282x2010000600004.
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Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the presynaptic neuromuscular transmission, which more frequently occurs as the remote effect of a neoplasm, in the paraneoplastic form (P-LEMS), or in a non-paraneoplastic form (NP-LEMS); but few studies describe the clinical features of NP-LEMS. We analyzed the clinical manifestations, laboratory findings, electrophysiological studies, and treatment responses in ten Brazilian patients suffering from NP-LEMS. The mean age was 41.5 years. More often neurological findings were hyporeflexia or areflexia with a post-exercise improvement. Treatment response occurred with pyridostigmine, guanidine, prednisone, azathioprine, and cyclosporine; but not response was observed after intravenous immunoglobulin and plasma exchange. Age at onset, clinical manifestations, and electrophysiological abnormalities can help more in the diagnosis than serum antibodies; the symptomatic treatment with pyridostigmine was effective; and the immunosuppressive treatment with prednisone, azathioprine, or cyclosporine was more beneficial than plasma exchange or intravenous immunoglobulin treatment.
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Klimašauskienė, A., and R. Bunevičiūtė. "Miastenija ir Lambert-Eaton miasteninis sindromas, jų gydymo rekomendacijos ir ypatumai COVID-19 pandemijos metu." Neurologijos seminarai 24, no. 84 (July 1, 2020): 138–44. http://dx.doi.org/10.29014/ns.2020.20.
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Miastenija ir Lambert-Eaton (LEMS) miasteninis sindromas yra autoimuninės neurologinės ligos, kurios pasireiškia raumenų silpnumu ir patologiniu nuovargiu. Miastenija yra dažniausia neuroraumeninė liga, o LEMS – labai retas susirgimas. Abi ligos simptomiškai gydomos acetilcholinesterazės inhibitoriais, LEMS – dar ir 3,4 diaminopiridinu ir amifampiridinu, o ilgalaikiam gydymui skiriami imunosupresiniai vaistai: gliukokortikoidai, azatioprinas, kiti imunosupresantai. Timektomija pacientams su timoma ir atrinktai grupei be timomos yra esminis gydymo metodas, taikomas sergant miastenija. Abiejų ligų paūmėjimai gydomi intraveniniais imunoglobulinais ar gydomosiomis aferezėmis. Miastenijos ir LEMS gydymą pandemijos metu rekomenduojama pradėti ir tęsti pagal bendras rekomendacijas. Jei pacientams skirta imunosupresantų, šis gydymas turėtų būti tęsiamas, nes nauda yra didesnė už galimą žalą. Gydymas imunosupresantais nutraukiamas tik išsivysčius sunkiai infekcijai.
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Xu, You-Fen, Sandra J. Hewett, and William D. Atchison. "Passive Transfer of Lambert-Eaton Myasthenic Syndrome Induces Dihydropyridine Sensitivity of I Ca in Mouse Motor Nerve Terminals." Journal of Neurophysiology 80, no. 3 (September 1, 1998): 1056–69. http://dx.doi.org/10.1152/jn.1998.80.3.1056.
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Xu, You-Fen, Sandra J. Hewett, and William D. Atchison. Passive transfer of Lambert-Eaton Myasthenic Syndrome induces dihydropyridine sensitivity of I Ca in mouse motor nerve terminals. J. Neurophysiol. 80: 1056–1069, 1998. Mice were injected for 30 days with plasma from three patients with Lambert-Eaton Myasthenic Syndrome (LEMS). Recordings were made from the perineurial sheath of motor axon terminals of triangularis sterni muscle preparations. The objective was to characterize pharmacologically the identity of kinetically distinct, defined potential changes associated with motor nerve terminal Ca2+ currents ( I Ca) that were affected by LEMS autoantibodies. I Ca elicited at 0.01 Hz were significantly reduced in amplitude by ∼35% of control in LEMS-treated nerve terminals. During 10-Hz stimulation, I Ca amplitude was unchanged in LEMS-treated motor nerve terminals, but was depressed in control. During 20- or 100-Hz trains, facilitation of I Ca occurred in LEMS-treated nerve terminals whereas in control, no facilitation occurred during the trains at 20 Hz and marked depression occurred at 100 Hz. Saturation for amplitude and duration of I Ca in control terminals occurred at 2 and 4–6 mM extracellular Ca2+, respectively; in LEMS-treated terminals, the extracellular Ca2+ concentration had to increase by two to three times of control to cause saturation. Amplitude of the two components of I Ca observed when the preparation was exposed to 50 μM 3,4-diaminopyridine and 1 mM tetraethylammonium were both reduced by LEMS plasma treatment. The fast component ( I Ca,f) was reduced by 35%, whereas the slow component ( I Ca,s) was reduced by 37%. ω-Agatoxin IVA (ω-Aga-IVA; 0.15 μM) and ω-conotoxin-MVIIC (ω-CTx-MVIIC; 5 μM) completely blocked I Ca in control motor nerve terminals. The same concentrations of toxins were 20–30% less effective in blocking I Ca in LEMS-treated terminals. The residual I Ca remaining after treatment with ω-Aga-IVA or ω-CTx-MVIIC was blocked by 10 μM nifedipine and 10 μM Cd2+. Thus LEMS plasma appears to downregulate ω-Aga-IVA-sensitive (P-type) and/or ω-CTx-MVIIC-sensitive (Q- type) Ca2+ channels in murine motor nerve terminals, whereas dihydropyridine (DHP)-sensitive (L-type) Ca2+ channels are unmasked in these terminals. Acute exposure (90 min) of rat forebrain synaptosomes to LEMS immunoglobulins (Igs; 4 mg/ml) did not alter the binding of [3H]-nitrendipine or [125I]-ω-conotoxin-GVIA (-ω-CgTx GVIA) when compared with synaptosomes incubated with an equivalent concentration of control Igs. Conversely, LEMS Igs significantly decreased the B max for [3H]-verapamil to ∼45% of control. The apparent affinity of verapamil ( K D) for the remaining receptors was not significantly altered. Thus acute exposure of isolated central nerve terminals to LEMS Igs does not increase DHP sensitivity, whereas it reduces the number of binding sites for verapamil but not for nitrendipine or ω-CgTx-GVIA. These results suggest that chronic but not acute exposure to LEMS Igs either upregulates or unmasks DHP-sensitive Ca2+ channels in motor nerve endings.
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Titulaer, Maarten J., Rinse Klooster, Marko Potman, Lidia Sabater, Francesc Graus, Ingrid M. Hegeman, Peter E. Thijssen, et al. "SOX Antibodies in Small-Cell Lung Cancer and Lambert-Eaton Myasthenic Syndrome: Frequency and Relation With Survival." Journal of Clinical Oncology 27, no. 26 (September 10, 2009): 4260–67. http://dx.doi.org/10.1200/jco.2008.20.6169.
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Purpose SOX1 antibodies are common in small-cell lung carcinoma (SCLC) with and without paraneoplastic syndrome (PNS) and can serve as serological tumor marker. Addition of other antibodies might improve its diagnostic power. We validated an enzyme-linked immunosorbent assay (ELISA) to assess the diagnostic value of serum antibodies in SCLC and Lambert-Eaton myasthenic syndrome (LEMS). Clinical outcome with respect to SOX antibodies was evaluated, as the SOX-related antitumor immune response might help to control the tumor growth. Patients and Methods We used recombinant SOX1, SOX2, SOX3, SOX21, HuC, HuD, or HelN1 proteins in an ELISA to titrate serum samples and validated the assay by western blot. We tested 136 consecutive SCLC patients, 86 LEMS patients (43 with SCLC), 14 patients with SCLC and PNS (paraneoplastic cerebellar degeneration or Hu syndrome), 62 polyneuropathy patients, and 18 healthy controls. Results Our ELISA was equally reliable as western blot. Forty-three percent of SCLC patients and 67% of SCLC-LEMS patients had antibodies to one of the SOX or Hu proteins. SOX antibodies had a sensitivity of 67% and a specificity of 95% to discriminate between LEMS with SCLC and nontumor LEMS. No difference in survival was observed between SOX positive and SOX negative SCLC patients. Conclusion SOX antibodies are specific serological markers for SCLC. Our assay is suitable for high throughput screening, detecting 43% of SCLC. SOX antibodies have diagnostic value in discriminating SCLC-LEMS from nontumor LEMS, but have no relation to survival in patients with SCLC.
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Komatsu, Teppei, Kota Bokuda, Toshio Shimizu, Tetsuo Komori, and Reiji Koide. "Pseudomyopathic Changes in Needle Electromyography in Lambert-Eaton Myasthenic Syndrome." Case Reports in Neurological Medicine 2013 (2013): 1–2. http://dx.doi.org/10.1155/2013/369278.
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Lambert-Eaton myasthenic syndrome (LEMS) is a rare presynaptic disorder of the neuromuscular junction in association with cancer and subsequently in cases in which no neoplasm has been detected (O’Neill et al., 1988). The diagnosis of LEMS is based on the combination of fluctuating muscle weakness, diminished or absent reflexes, and a more than 60% increment of compound muscle action potential (CMAP) amplitude after brief exercise or 50 Hz stimulation for 1 s in a repetitive nerve stimulation (RNS) test (Oh et al., 2005). On the other hand, needle electromyography (EMG) findings related to LEMS have not been well described. Here, we report a case of LEMS, which showed apparent myopathic changes in needle EMG findings. Furthermore, we retrospectively examined the needle EMG findings in 8 patients with LEMS. In six of the 8 patients, the EMG findings showed myopathy-like findings. Although the findings of needle EMG indicated myopathic changes at a glance, the motor unit potential (MUP) returned to normal after a sustained strong muscle contraction. We propose the name “pseudomyopathic changes” for this phenomenon.
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Kostmann, Michael, and Wolfgang K. Härdle. "Forecasting in Blockchain-Based Local Energy Markets." Energies 12, no. 14 (July 16, 2019): 2718. http://dx.doi.org/10.3390/en12142718.
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Increasingly volatile and distributed energy production challenges traditional mechanisms to manage grid loads and price energy. Local energy markets (LEMs) may be a response to those challenges as they can balance energy production and consumption locally and may lower energy costs for consumers. Blockchain-based LEMs provide a decentralized market to local energy consumer and prosumers. They implement a market mechanism in the form of a smart contract without the need for a central authority coordinating the market. Recently proposed blockchain-based LEMs use auction designs to match future demand and supply. Thus, such blockchain-based LEMs rely on accurate short-term forecasts of individual households’ energy consumption and production. Often, such accurate forecasts are simply assumed to be given. The present research tested this assumption by first evaluating the forecast accuracy achievable with state-of-the-art energy forecasting techniques for individual households and then, assessing the effect of prediction errors on market outcomes in three different supply scenarios. The evaluation showed that, although a LASSO regression model is capable of achieving reasonably low forecasting errors, the costly settlement of prediction errors can offset and even surpass the savings brought to consumers by a blockchain-based LEM. This shows that, due to prediction errors, participation in LEMs may be uneconomical for consumers, and thus, has to be taken into consideration for pricing mechanisms in blockchain-based LEMs.
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Franzoi, Ana Cristina, Ana Luiza Baptista, Ana Maria Carvalho, Wagner Gonçalves, Anna Christina Boari Rosa, Alexandre Pinto, and Kátia Regina Carvalho. "Perfil funcional de locomoção em um grupo de pacientes com lesão medular atendidos em um centro de reabilitação." Coluna/Columna 8, no. 4 (December 2009): 401–7. http://dx.doi.org/10.1590/s1808-18512009000400010.
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OBJETIVO: descrever o perfil de locomoção de um grupo de pacientes com lesão medular (LM), correlacionando-o com as características da amostra. MÉTODOS: setenta pacientes (50 com LM traumática e 20 com LM não-traumática). Instrumentos: Classificação Funcional da Marcha Modificada (CFMM), WISCI-II, Escala de Ashworth Modificada (EAM), Classificação AIS, Lower Extremities Muscle Score (AIS-LEMS). Estatística: descritiva, comparação entre grupos, testes de correlação considerando p<0,05. RESULTADOS: a média da idade dos pacientes foi de 36 anos; 72,9% dos pacientes eram homens; tempo médio de lesão: 4,7 anos; AIS: 38,6% A, 10% B, 22,8% C, 28,6% D; Nível de lesão: 32,8% cervical, 52,9% torácico, 14,3% lombossacro. Causas mais frequentes: PAF 30%; acidente automobilístico 18,6%; CFMM: CR 34,3%; marcha terapêutica 20%; domiciliar 12,8%; comunitária 32,9%. WISCI-II: 7,5. AIS-LEMS: 13,4. Houve diferença entre os grupos LM traumática e não-traumática quanto à idade, sexo, AIS, AIS-LEMS, CFMM e WISCI-II. Correlações significativas: AIS correlacionou-se positivamente com CFMM, WISCI-II, AIS-LEMS. Nível de lesão correlacionado negativamente com EAM. AIS-LEMS positivamente com a AIS, CFMM, WISCI-II e negativamente com apoio e órteses na deambulação. O tempo de lesão e a presença de dor incapacitante não se correlacionaram com as variáveis estudadas. CONCLUSÕES: o grupo de LM traumática era composto por pacientes mais jovens e com maior prevalência de lesão completa. A idade se correlacionou com o uso de aparelhos de auxílio à marcha e órteses. O desempenho funcional da marcha esteve ligado à AIS e ao AIS-LEMS, sendo que a marcha comunitária necessitou de um AIS-LEMS de pelo menos 32 pontos.
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Titulaer, Maarten J., Paul Maddison, Jacob K. Sont, Paul W. Wirtz, David Hilton-Jones, Rinse Klooster, Nick Willcox, et al. "Clinical Dutch-English Lambert-Eaton Myasthenic Syndrome (LEMS) Tumor Association Prediction Score Accurately Predicts Small-Cell Lung Cancer in the LEMS." Journal of Clinical Oncology 29, no. 7 (March 1, 2011): 902–8. http://dx.doi.org/10.1200/jco.2010.32.0440.
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Purpose Approximately one half of patients with Lambert-Eaton myasthenic syndrome (LEMS) have small-cell lung carcinomas (SCLC), aggressive tumors with poor prognosis. In view of its profound impact on therapy and survival, we developed and validated a score to identify the presence of SCLC early in the course of LEMS. Patients and Methods We derived a prediction score for SCLC in LEMS in a nationwide cohort of 107 Dutch patients, and validated it in a similar cohort of 112 British patients. A Dutch-English LEMS Tumor Association Prediction (DELTA-P) score was developed based on multivariate logistic regression. Results Age at onset, smoking behavior, weight loss, Karnofsky performance status, bulbar involvement, male sexual impotence, and the presence of Sry-like high-mobility group box protein 1 serum antibodies were independent predictors for SCLC in LEMS. A DELTA-P score was derived allocating 1 point for the presence of each of the following items at or within 3 months from onset: age at onset ≥ 50 years, smoking at diagnosis, weight loss ≥ 5%, bulbar involvement, erectile dysfunction, and Karnofsky performance status lower than 70. The area under the curve of the receiver operating curve was 94.4% in the derivation cohort and 94.6% in the validation set. A DELTA-P score of 0 or 1 corresponded to a 0% to 2.6% chance of SCLC, whereas scores of 4, 5, and 6 corresponded to chances of SCLC of 93.5%, 96.6%, and 100%, respectively. Conclusion The simple clinical DELTA-P score discriminated patients with LEMS with and without SCLC with high accuracy early in the course of LEMS.
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Khashan, Morsi, Micheal Raad, Mostafa H. El Dafrawy, Varun Puvanesarajah, and Khaled M. Kebaish. "Postoperative changes in neurological function after 3-column osteotomy: risk factor analysis of 199 patients." Journal of Neurosurgery: Spine 30, no. 5 (May 2019): 568–73. http://dx.doi.org/10.3171/2018.11.spine18698.
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OBJECTIVEThe authors evaluated the neurological outcomes of adult spinal deformity patients after 3-column osteotomy (3CO), including severity and long-term improvement of neurological complications, as well as risk factors for neurological deficit at 1 year postoperatively. Although 3CO is effective for correcting rigid spinal deformity, it is associated with a high complication rate. Neurological deficits, in particular, cause disability and dissatisfaction.METHODSThe authors retrospectively queried a prospective database of adult spinal deformity patients who underwent vertebral column resection or pedicle subtraction osteotomy between 2004 and 2014 by one surgeon at a tertiary care center. The authors included 199 adults with at least 1-year follow-up. The primary outcome measure was change in lower-extremity motor scores (LEMSs), which were obtained preoperatively, within 2 weeks postoperatively, and at 6 and 12 months postoperatively. To identify risk factors for persistent neurological deficit, the authors compared patient and surgical characteristics with a declined LEMS at 12-month follow-up (n = 10) versus those with an improved/maintained LEMS at 12-month follow-up (n = 189).RESULTSAt the first postoperative assessment, the LEMS had improved in 15% and declined in 10% of patients compared with preoperative scores. At the 6-month follow-up, 6% of patients continued to have a decline in LEMS, and 16% had improvement. At 12 months, LEMS had improved in 17% and declined in 5% of patients compared with preoperative scores. The only factor significantly associated with a decline in 12-month LEMS was high-grade spondylolisthesis as an indication for surgery (OR 13, 95% CI 3.2–56).CONCLUSIONSAlthough the LEMS declined in 10% of patients immediately after 3CO, at 12 months postoperatively, only 5% of patients had neurological motor deficits. A surgical indication of high-grade spondylolisthesis was the only factor associated with neurological deficit at 12 months postoperatively.
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Yoon, Connie H., Jocelyn Owusu-Guha, Adam Smith, and Pamela Buschur. "Amifampridine for the Management of Lambert-Eaton Myasthenic Syndrome: A New Take on an Old Drug." Annals of Pharmacotherapy 54, no. 1 (July 18, 2019): 56–63. http://dx.doi.org/10.1177/1060028019864574.
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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, TRIP database, and EMBASE searches were conducted without a back date (current to June 2019) utilizing the following search terms: amifampridine, 3,4-diaminopyridine, and Lambert-Eaton myasthenic syndrome. Completed trials were also reviewed at clinicaltrials.gov. Study Selection and Data Extraction: Criteria for article inclusion consisted of human subjects, age ≥18 years, phase II or III clinical trials, and English language for both drugs. Observational and pharmacokinetic studies for amifampridine were also included. Data Synthesis: Prior to the approval of amifampridine, 3,4-DAP was first-line for the management of LEMS symptoms. Two phase III trials have evaluated amifampridine to confirm efficacy, both showing superiority over placebo in the management of LEMS symptoms, with minimal adverse effects. A significant improvement in both quantitative myasthenia gravis scores and Subjective Global Impression scores was established at days 4 and 14. Relevance to Patient Care and Clinical Practice: With an improved stability profile and decreased dose variability, amifampridine will likely assume the role of first-line management of LEMS. Conclusions: Amifampridine has been shown to improve symptoms of LEMS and is generally well tolerated.
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Arimbawa, Putu, Kliwon Hidayat, Edi Dwi Cahyono, Yayuk Yuliati, and L. Daud. "Peran Komunikasi dalam Kelembagaan Petani pada Pengembangan Kakao di Kabupaten Kolaka Timur." Jurnal Ilmiah Membangun Desa dan Pertanian 5, no. 1 (January 21, 2020): 8. http://dx.doi.org/10.37149/jimdp.v5i1.10195.
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The purpose of this study is to analyze the role of communication in the information management process that occurs in farmer institutions to support cocoa development. This research is a survey research with a questionnaire as a data collection tool. The research location is in East Kolaka Regency with the target of LEMS cocoa farmers. The research location is in East Kolaka Regency with the target of LEMS cocoa farmers. Sampling as many as 267 farmers spread over 18 LEMS by proportional random sampling. Descriptive research data analysis. The results showed that the role of communication in LEMS institutions was in the medium category cocoa development. The role of communication that needs to be improved is the role in accessing information and mediating information. Information management is still dominantly sourced from explicit knowledge and does not accommodate knowledge from farmers' experience (tacit knowledge).
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Agrawal, Kavita, and Nirav Agrawal. "Lambert-Eaton Myasthenic Syndrome Secondary to Nivolumab and Ipilimumab in a Patient with Small-Cell Lung Cancer." Case Reports in Neurological Medicine 2019 (July 2, 2019): 1–4. http://dx.doi.org/10.1155/2019/5353202.
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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. After 4 months of starting immunotherapy, he reported extreme fatigue, muscular weakness, and poor appetite. He was diagnosed with hypothyroidism, primary adrenal insufficiency, and Lambert-Eaton Myasthenic Syndrome (LEMS). LEMS can be both a paraneoplastic syndrome of SCLC and an adverse effect of immunotherapy. Currently, there is no diagnostic test available to determine if a case of LEMS is a paraneoplastic syndrome or immunotherapy-related adverse effect. In our patient, we felt that LEMS was an immunotherapy-related adverse effect rather being a paraneoplastic syndrome. Our determination was based on the time of onset of muscular weakness, presence of other immunotherapy-mediated adverse events, and the appearance of symptoms in spite of SCLC that had been stabilized on immunotherapy. Accordingly, immunotherapy was stopped and a brief tapering course of steroids was initiated. Our patient’s muscular weakness from LEMS responded well. His clinical improvement persisted even with radiologic progression of disease after cessation of immunotherapy.
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Sadalage, Girija, Sajjad Ali, Paul Mocroft, Adrian Williams, and Saiju Jacob. "UNUSUAL DECREMENTAL RESPONSE IN LAMBERT EATON MYASTHENIC SYNDROME." Journal of Neurology, Neurosurgery & Psychiatry 86, no. 11 (October 14, 2015): e4.50-e4. http://dx.doi.org/10.1136/jnnp-2015-312379.143.
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Lambert Eaton Myasthenic Syndrome (LEMS) is an autoimmune disorder affecting the neuromuscular junction caused by antibodies against voltage gated calcium channel antibodies. The neurophysiological hallmark of LEMS is incremental response to repetitive nerve stimulation, especially at high frequency stimulation.A 54-year-old gentleman presented to the Neurology clinic with a 9-month history of progressive walking difficulty. He had difficulty standing up from a sitting down position and the symptoms were worse towards the end of the day. He had a transient history of dry mouth when the symptoms started, but denied any postural dizziness, sweating or bowel/bladder symptoms. He denied having any double vision, droopy eyelids, speech or swallowing symptoms. He had lost 1.5 stones in weight over the preceding 7–8 months. Examination showed proximal muscle weakness with reduced reflexes, which could not be potentiated. A clinical suspicion of LEMS was made, but the neurophysiological examination (repeated on three occasions) showed consistently decremental response at low and high frequency stimulations.The VGCC antibody was positive (293, normal 0–45), confirming the clinical suspicion of LEMS. The AChR and MuSK antibodies were negative.This is an unusual neurophysiological finding of a decremental response in a patient with clinical and immunological evidence of LEMS.
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Rattananan, Watcharasam, Mohammad Alsharabati, and Shin J. Oh. "Ocular LEMS or MLOS." Muscle & Nerve 54, no. 5 (June 9, 2016): 981–82. http://dx.doi.org/10.1002/mus.25189.
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Troyanova-Slavkova, S., C. Seidl, P. Bortkevicius, L. Eickenscheidt, and L. Kowalzick. "Ein seltener Fall von Lambert-Eaton-Myasthenischem-Syndrom assoziiert mit Merkelzell-Karzinom und präexistenter Vitiligo." Aktuelle Dermatologie 44, no. 03 (December 5, 2017): 109–13. http://dx.doi.org/10.1055/s-0043-120252.
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ZusammenfassungDas Lambert-Eaton-Myasthenische-Syndrom (LEMS) ist eine sehr seltene neurologische Autoimmunerkrankung, für die eine proximal betonte Muskelschwäche charakteristisch ist. Die Erkrankung ist als paraneoplastisches neurologisches Symptom bekannt, das besonders mit neuroendokrinen Tumoren assoziiert ist und v. a. beim kleinzelligen Lungenkarzinom auftreten kann. Das Merkelzell-Karzinom (MCC) ist eine seltene Malignität an der Haut, die eine Tendenz für lokale Rezidive und regionale Lymphknotenmetastasen hat. Es tritt v. a. bei älteren Patienten an den sonnenexponierten Hautarealen als schnell wachsendes intrakutanes Knötchen auf. Hier stellen wir einen Fall von MCC vor, das bei einem vergleichsweise jungen 40-jährigen Patienten mit LEMS und Vitiligo diagnostiziert wurde. Nach einer Überprüfung der Literatur zeigt sich das Auftreten von LEMS mit MCC und Vitiligo als eine Rarität.
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Ashjazadeh, Nahid, Maryam Sharifian Dorche, Soheila Zareifar, Masood Nomovi, and Amirhossein Sharian Dorche. "Rare Coexistence of Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome with Adenocarcinoma of the Bladder: A Case Report." Internal Medicine and Medical Investigation Journal 2, no. 4 (October 5, 2017): 171. http://dx.doi.org/10.24200/imminv.v2i4.71.
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Abstract Introduction: The coexistence of myasthenia gravis and Lambert-Eaton myasthenic syndrome (LEMS) is very rare.Case report: Herein we report an 82 year old man a known case of adenocarcinoma of bladder who came with ptosis in left eye, diplopia, and dysphagia, weakness of both lower extremities and autonomic dysfunction. According to history, physical examination and para clinical work ups coexisting of myasthenia gravis and LEMS was diagnosed.Discussion: our patient is the first patient with Overlap myasthenic syndrome on the base of adenocarcinoma of bladder. The purpose of this case report is to emphasize on this fact that a patient with LEMS should be evaluate carefully for detecting underlying malignancies. Although in the most cases the origin is lung, extra pulmonary origin such as urinary system should be evaluate carefully too.
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Cerpa, Meghan, Lawrence G. Lenke, Michael G. Fehlings, Christopher I. Shaffrey, Kenneth M. C. Cheung, and Leah Yacat Carreon. "Evolution and Advancement of Adult Spinal Deformity Research and Clinical Care: An Overview of the Scoli-RISK-1 Study." Global Spine Journal 9, no. 1_suppl (May 2019): 8S—14S. http://dx.doi.org/10.1177/2192568219828729.
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Study Design: Narrative review. Objective: The prevalence of adult spinal deformity (ASD) has been cited anywhere between 2-32%, while the prevalence in the elderly population has been estimated at 68%. Neurologic complications following ASD surgery remains a concern. Previous literature reported incidence of neurologic complications varied between 1-10%, while non-neurologic complications reported were as high as 50%. To assess the incidence of neurologic deficits, complications, and outcomes following ASD surgery, an international group of spine deformity surgeons initiated a prospective, multicenter, international, observational study: Scoli-RISK-1. Methods: Two hundred seventy-two patients were enrolled from 15 centers with ASD having primary or revision surgery with a major Cobb≥80°, revision including an osteotomy, and/or a complex 3-column osteotomy. Patients had lower extremity muscle strength (LEMS) exams performed preoperatively and at specific time points through 2-year follow-up. Results: Preoperatively, 203 patients (74.9%) had no LEMS impairment (normal) and 68 (25.1%) had a LEMS of <50 (abnormal). Compared with baseline, 23.0% of all patients experienced a LEMS decline at discharge, with this rate decreasing to 17.1% at 6-weeks and to 9.9% at 6-months and remaining stable at 10.0% at 2-years. Conclusion: This study revealed that a decline in LEMS after complex ASD surgery is common and more frequent than previously reported. We identified such a decline in 23.0% of patients at discharge, with neurologic function recovering over time to a decline of 10.0% at 2-years postoperatively. The Scoli-RISK-1 study revealed valuable information regarding the incidence, natural history, and prognosis of neurologic and non-neurologic complications following ASD surgery and provides useful information for patient counseling.
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Campforts, Benjamin, Wolfgang Schwanghart, and Gerard Govers. "Accurate simulation of transient landscape evolution by eliminating numerical diffusion: the TTLEM 1.0 model." Earth Surface Dynamics 5, no. 1 (January 18, 2017): 47–66. http://dx.doi.org/10.5194/esurf-5-47-2017.
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Abstract. Landscape evolution models (LEMs) allow the study of earth surface responses to changing climatic and tectonic forcings. While much effort has been devoted to the development of LEMs that simulate a wide range of processes, the numerical accuracy of these models has received less attention. Most LEMs use first-order accurate numerical methods that suffer from substantial numerical diffusion. Numerical diffusion particularly affects the solution of the advection equation and thus the simulation of retreating landforms such as cliffs and river knickpoints. This has potential consequences for the integrated response of the simulated landscape. Here we test a higher-order flux-limiting finite volume method that is total variation diminishing (TVD-FVM) to solve the partial differential equations of river incision and tectonic displacement. We show that using the TVD-FVM to simulate river incision significantly influences the evolution of simulated landscapes and the spatial and temporal variability of catchment-wide erosion rates. Furthermore, a two-dimensional TVD-FVM accurately simulates the evolution of landscapes affected by lateral tectonic displacement, a process whose simulation was hitherto largely limited to LEMs with flexible spatial discretization. We implement the scheme in TTLEM (TopoToolbox Landscape Evolution Model), a spatially explicit, raster-based LEM for the study of fluvially eroding landscapes in TopoToolbox 2.
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Jones, R. "Lambert-Eaton Myasthenic Syndrome: LEMS." Clinical Neurophysiology 119 (October 2008): S152. http://dx.doi.org/10.1016/s1388-2457(08)60554-4.
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Behrman, Andrea L., Preeti M. Nair, Mark G. Bowden, Robert C. Dauser, Benjamin R. Herget, Jennifer B. Martin, Chetan P. Phadke, et al. "Locomotor Training Restores Walking in a Nonambulatory Child With Chronic, Severe, Incomplete Cervical Spinal Cord Injury." Physical Therapy 88, no. 5 (May 1, 2008): 580–90. http://dx.doi.org/10.2522/ptj.20070315.
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Background and Purpose Locomotor training (LT) enhances walking in adult experimental animals and humans with mild-to-moderate spinal cord injuries (SCIs). The animal literature suggests that the effects of LT may be greater on an immature nervous system than on a mature nervous system. The purpose of this study was to evaluate the effects of LT in a child with chronic, incomplete SCI. Subject The subject was a nonambulatory 4½-year-old boy with an American Spinal Injury Association Impairment Scale (AIS) C Lower Extremity Motor Score (LEMS) of 4/50 who was deemed permanently wheelchair-dependent and was enrolled in an LT program 16 months after a severe cervical SCI. Methods A pretest-posttest design was used in the study. Over 16 weeks, the child received 76 LT sessions using both treadmill and over-ground settings in which graded sensory cues were provided. The outcome measures were ASIA Impairment Scale score, gait speed, walking independence, and number of steps. Result One month into LT, voluntary stepping began, and the child progressed from having no ability to use his legs to community ambulation with a rolling walker. By the end of LT, his walking independence score had increased from 0 to 13/20, despite no change in LEMS. The child's final self-selected gait speed was 0.29 m/s, with an average of 2,488 community-based steps per day and a maximum speed of 0.48 m/s. He then attended kindergarten using a walker full-time. Discussion and Conclusion A simple, context-dependent stepping pattern sufficient for community ambulation was recovered in the absence of substantial voluntary isolated lower-extremity movement in a child with chronic, severe SCI. These novel data suggest that some children with severe, incomplete SCI may recover community ambulation after undergoing LT and that the LEMS cannot identify this subpopulation.
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Fiana, Dewi Nur, Sunaryo B. Sastradimaja, and Badai Bhatara Tiksnadi. "The Differences requency of Lower Extremities Muscle Strength and Functional Capacity on the difference Supervised Exercise Frequency in Post Cardiac Bypass Graft Surgery." Indonesian Journal of Physical Medicine & Rehabilitation 6, no. 01 (June 1, 2017): 34. http://dx.doi.org/10.36803/ijpmr.v6i01.150.
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Introduction: The optimal time to do exercise in adaptation phase was 36 to 72 hours. Patients with cardiovasculardisease may have an extended adaptation phase to 96 hours and above. It was necessary to know the mosteffective supervised exercise in phase II cardiac rehabilitation between three times/week for 36-hours, and twotimes/week for 96-hours.Method: This study involved 30 patients post-Cardiac Bypass Graft Surgery (CABG) participated in phase IIcardiac rehabilitation at Hasan Sadikin Hospital, Bandung. Subjects divided into two groups; that have done twotimes/week (group 1) and three times/week of the supervised exercised (group 2) for four weeks. The Lowerextremity muscle strength (LEMS) examined by conducting a chair standing test, while functional capacity (FC)evaluated by the 6-minute walking test.Result:Subjects were 58,54±5,90 y.o (group 1) and 61,66±6,36(group 2). The FC and the LEMS before andafter exercise were 10,98 and 15,96 ml/kg(<0,001) on the FC; 7,8 and 12,9 times (<0,001) on the LEMS in group1, besides 9,6 and 14,9 ml/kg(<0,001) on the FC; 8,7 and 13,0 times (<0,001) on group 2,Conclusion. Both groups have increased of the FC and the LEMS after exercise. There were no differencebetween 2 times and 3 times of supervised exercises a week on post CABG pasientsKeywords: coronary artery bypass graft, the frequency of exercise, functional capacity, muscle strength =
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Lorenzoni, Paulo José, Cláudia Suemi Kamoi Kay, Lineu Cesar Werneck, and Rosana Herminia Scola. "Lambert-Eaton myasthenic syndrome: the 60th anniversary of Eaton and Lambert's pioneering article." Arquivos de Neuro-Psiquiatria 76, no. 2 (February 2018): 124–26. http://dx.doi.org/10.1590/0004-282x20170194.
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ABSTRACT 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.
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Oh, Shin J., and Jörn Peter Sieb. "Update on Amifampridine as a Drug of Choice in Lambert-Eaton Myasthenic Syndrome." US Neurology 10, no. 02 (2014): i. http://dx.doi.org/10.17925/usn.2014.10.02.i.
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Lambert-Eaton myasthenic syndrome (LEMS) is a disabling autoimmune disorder involving impairment of neuromuscular transmission and producing serious muscle weakness, for which few effective medications are currently available. 3,4-diaminopyridine (3,4-DAP, INN/USAN: amifampridine) is the leading treatment for LEMS and has been available for over 25 years as an unapproved drug under treatment and expanded access protocols filed with the US Food and Drug Administration (FDA) or from compounding pharmacies in the US. Administering the correct dose of 3,4-DAP is criticaloverdosing can increase the risk for seizures and other adverse events, while underdosing can result in a substantial loss of efficacy or even treatment failure. Two recent studies have shown a wide variation in the 3,4-DAP content of compounded preparations. A tablet formulation of 3,4-DAP phosphate salt (FIRDAPSETM) has been licenced in Europe with orphan medicinal product status since 2009 and appears to be as efficacious as the base in relieving the symptoms of LEMS. The product has also received orphan drug status in the US and is currently being evaluated in a multicenter, double-blind, placebo-controlled phase III trial to support New Drug Application (NDA) approval in the US. A recent safety trial in healthy volunteers using doses at and above normal levels has shown no effect on QT intervals, heart rate, or cardiac depolarization. Based on available clinical trial data, amifampridine phosphate was recently given Breakthrough Therapy designation by the FDA, which may enable fast-track NDA approval, thus increasing the potential for more patients with LEMS to receive an effective therapy.
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Mareev, Evgenii, Nikita Minaev, Vyacheslav Zhigarkov, and Vladimir Yusupov. "Evolution of Shock-Induced Pressure in Laser Bioprinting." Photonics 8, no. 9 (September 7, 2021): 374. http://dx.doi.org/10.3390/photonics8090374.
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Laser bioprinting with gel microdroplets that contain living cells is a promising method for use in microbiology, biotechnology, and medicine. Laser engineering of microbial systems (LEMS) technology by laser-induced forward transfer (LIFT) is highly effective in isolating difficult-to-cultivate and uncultured microorganisms, which are essential for modern bioscience. In LEMS the transfer of a microdroplet of a gel substrate containing living cell occurs due to the rapid heating under the tight focusing of a nanosecond infrared laser pulse onto thin metal film with the substrate layer. During laser transfer, living organisms are affected by temperature and pressure jumps, high dynamic loads, and several others. The study of these factors’ role is important both for improving laser printing technology itself and from a purely theoretical point of view in relation to understanding the mechanisms of LEMS action. This article presents the results of an experimental study of bubbles, gel jets, and shock waves arising in liquid media during nanosecond laser heating of a Ti film obtained using time-resolving shadow microscopy. Estimates of the pressure jumps experienced by microorganisms in the process of laser transfer are performed: in the operating range of laser energies for bioprinting LEMS technology, pressure jumps near the absorbing film of the donor plate is about 30 MPa. The efficiency of laser pulse energy conversion to mechanical post-effects is about 10%. The estimates obtained are of great importance for microbiology, biotechnology, and medicine, particularly for improving the technologies related to laser bioprinting and the laser engineering of microbial systems.
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Reddel, Stephen. "1. How not to miss LEMS." Clinical Neurophysiology 121, no. 4 (April 2010): e1. http://dx.doi.org/10.1016/j.clinph.2009.10.021.
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Winkel, Antony, Katrina Reardon, and Leslie Roberts. "100 Autoimmune lambert eaton myasthenic syndrome in young adults." Journal of Neurology, Neurosurgery & Psychiatry 89, no. 6 (May 24, 2018): A39.3—A40. http://dx.doi.org/10.1136/jnnp-2018-anzan.99.
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IntroductionLambert Eaton myasthenic syndrome (LEMS) is a neuromuscular junction transmission disorder that is associated with malignancy (most often small cell lung cancer) and antibodies to the voltage gated calcium channel. Rarely, it can occur as a non-paraneoplastic phenomenon. The diagnosis can be difficult, and reduced motor amplitudes on nerve conduction studies can be misattributed to neurogenic or myopathic processes unless the referring physician or neurophysiologist considers LEMS in the differential diagnosis. Two cases of non-paraneoplastic LEMS were identified from testing performed in the Neurophysiology laboratory of St Vincent’s Hospital Melbourne in 2017. The histories, examination findings, investigations, extensive neurophysiological testing and response to treatment are presented.CasesThe two patients identified were aged 18 and 34 years at diagnosis. Both had a history of slowly progressive proximal weakness preferentially affecting the lower limbs over several years, with associated autonomic symptoms. The younger patient had become wheelchair dependent at age 17. Detailed neurophysiological testing demonstrated a pre-synaptic defect of neuromuscular junction transmission. The younger patient showed myopathic features on needle EMG. Voltage gated calcium channel antibodies were positive in both cases. Treatment with intravenous immunoglobulin, 3, 4-diaminopyridine, pyridostigmine, and azathioprine was associated with a definite improvement in strength and function in both patients. The patient with myopathic EMG features has residual moderate weakness but is now ambulant. Extensive screening for malignancies (including whole-body PET) has been negative, but surveillance will be continued for up to 5 years.ConclusionNon-paraneoplastic LEMS is a rare, but treatable, condition that warrants consideration, particularly when neurophysiological testing reveals reduced motor amplitudes. Successful diagnosis requires a threshold of suspicion in the neurophysiologist or referring neurologist.
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Manella, Kathleen J., Kathryn E. Roach, and Edelle C. Field-Fote. "Operant conditioning to increase ankle control or decrease reflex excitability improves reflex modulation and walking function in chronic spinal cord injury." Journal of Neurophysiology 109, no. 11 (June 1, 2013): 2666–79. http://dx.doi.org/10.1152/jn.01039.2011.
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Ankle clonus is common after spinal cord injury (SCI) and is attributed to loss of supraspinally mediated inhibition of soleus stretch reflexes and maladaptive reorganization of spinal reflex pathways. The maladaptive reorganization underlying ankle clonus is associated with other abnormalities, such as coactivation and reciprocal facilitation of tibialis anterior (TA) and soleus (SOL), which contribute to impaired walking ability in individuals with motor-incomplete SCI. Operant conditioning can increase muscle activation and decrease stretch reflexes in individuals with SCI. We compared two operant conditioning-based interventions in individuals with ankle clonus and impaired walking ability due to SCI. Training included either voluntary TA activation (TA↑) to enhance supraspinal drive or SOL H-reflex suppression (SOL↓) to modulate reflex pathways at the spinal cord level. We measured clonus duration, plantar flexor reflex threshold angle, timed toe tapping, dorsiflexion (DF) active range of motion, lower extremity motor scores (LEMS), walking foot clearance, speed and distance, SOL H-reflex amplitude modulation as an index of reciprocal inhibition, presynaptic inhibition, low-frequency depression, and SOL-to-TA clonus coactivation ratio. TA↑ decreased plantar flexor reflex threshold angle (−4.33°) and DF active range-of-motion angle (−4.32°) and increased LEMS of DF (+0.8 points), total LEMS of the training leg (+2.2 points), and nontraining leg (+0.8 points), and increased walking foot clearance (+ 4.8 mm) and distance (+12.09 m). SOL↓ decreased SOL-to-TA coactivation ratio (−0.21), increased nontraining leg LEMS (+1.8 points), walking speed (+0.02 m/s), and distance (+6.25 m). In sum, we found increased voluntary control associated with TA↑ outcomes and decreased reflex excitability associated with SOL↓ outcomes.
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Newsom-Davis, John. "Autoantibody-Mediated Channelopathies at the Neuromuscular Junction." Neuroscientist 3, no. 5 (September 1997): 337–46. http://dx.doi.org/10.1177/107385849700300515.
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The neuromuscular junction is vulnerable to antibody-mediated autoimmune attack, probably because it lacks the protection of the blood-brain barrier. This review focuses on three disorders: myasthenia gravis (MG) and the Lambert-Eaton myasthenic syndrome (LEMS), in both of which there is fatiguable muscle weakness, and acquired neuromyotonia (ANMT), in which hyperexcitable peripheral nerves lead to continuous muscle fiber activity and sometimes parasthesias. Each can occur as a paraneoplastic disorder (thymoma in MG and ANMT, and small cell lung cancer in LEMS). The clinical abnormalities are improved following plasmapheresis (which removes circulating antibodies), and injection of experimental animals with immunoglobulins of patients transfers the pathophysiological changes. The ion channel targets in these three disorders are the muscle acetylcholine receptor (a ligand-gated cation channel) in MG, nerve terminal and autonomic voltage-gated calcium channels in LEMS, and peripheral nerve voltage-gated potassium channels in ANMT. The autoantibody attack results in a reduced number of functional channels. Each of the autoantibodies can be detected in serum by immunoassay. These discoveries have allowed new approaches to treatment and suggest that there may be other undiscovered antibody-mediated ion channelopathies. NEUROSCIENTIST 3:337–346, 1997
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Altintas, Ayse, Justina Dargvainiene, Christiane Schneider-Gold, Nasrin Asgari, Ilya Ayzenberg, Andrea I. Ciplea, Ralf Junker, Frank Leypoldt, Klaus-Peter Wandinger, and Kerstin Hellwig. "Gender issues of antibody-mediated diseases in neurology: (NMOSD/autoimmune encephalitis/MG)." Therapeutic Advances in Neurological Disorders 13 (January 2020): 175628642094980. http://dx.doi.org/10.1177/1756286420949808.
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Neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AE), myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are antibody-mediated neurological diseases. They have mostly female predominance, affecting many women during childbearing age. Interactions between the underlying disease (or necessary treatment) and pregnancy can occur in every of these illnesses. Herein, we present the characteristics of NMOSD, AE, MG and LEMS in general, and review published data regarding the influence of the different diseases on fertility, pregnancy, puerperium, treatment strategy during pregnancy and post-partum period, and menopause but also male factors. We summarise key elements that should be borne in mind when confronted with such cases.
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Tiuryn, Jerzy, and Ewa Szczurek. "Learning signaling networks from combinatorial perturbations by exploiting siRNA off-target effects." Bioinformatics 35, no. 14 (July 2019): i605—i614. http://dx.doi.org/10.1093/bioinformatics/btz334.
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Abstract Motivation Perturbation experiments constitute the central means to study cellular networks. Several confounding factors complicate computational modeling of signaling networks from this data. First, the technique of RNA interference (RNAi), designed and commonly used to knock-down specific genes, suffers from off-target effects. As a result, each experiment is a combinatorial perturbation of multiple genes. Second, the perturbations propagate along unknown connections in the signaling network. Once the signal is blocked by perturbation, proteins downstream of the targeted proteins also become inactivated. Finally, all perturbed network members, either directly targeted by the experiment, or by propagation in the network, contribute to the observed effect, either in a positive or negative manner. One of the key questions of computational inference of signaling networks from such data are, how many and what combinations of perturbations are required to uniquely and accurately infer the model? Results Here, we introduce an enhanced version of linear effects models (LEMs), which extends the original by accounting for both negative and positive contributions of the perturbed network proteins to the observed phenotype. We prove that the enhanced LEMs are identified from data measured under perturbations of all single, pairs and triplets of network proteins. For small networks of up to five nodes, only perturbations of single and pairs of proteins are required for identifiability. Extensive simulations demonstrate that enhanced LEMs achieve excellent accuracy of parameter estimation and network structure learning, outperforming the previous version on realistic data. LEMs applied to Bartonella henselae infection RNAi screening data identified known interactions between eight nodes of the infection network, confirming high specificity of our model and suggested one new interaction. Availability and implementation https://github.com/EwaSzczurek/LEM Supplementary information Supplementary data are available at Bioinformatics online.
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Strupp, Michael, Julian Teufel, Andreas Zwergal, Roman Schniepp, Kamran Khodakhah, and Katharina Feil. "Aminopyridines for the treatment of neurologic disorders." Neurology: Clinical Practice 7, no. 1 (November 10, 2016): 65–76. http://dx.doi.org/10.1212/cpj.0000000000000321.
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AbstractPurpose of review:To identify the different indications for the treatment of neurologic disorders with the potassium channel blockers 4-aminopyridine (4-AP) and 3,4-diaminopyridine (3,4-DAP).Recent findings:4-AP is an effective symptomatic treatment for downbeat nystagmus (DBN), episodic ataxia type 2 (EA2) (5–10 mg TID), and impaired gait in multiple sclerosis (MS) (10 mg BID). 3,4-DAP (5 mg/d–20 mg TID) improves symptoms in Lambert-Eaton myasthenic syndrome (LEMS) (randomized placebo-controlled trials for all 4 entities). 4-AP may also be effective in cerebellar gait ataxia of different etiologies (2 case series), upbeat nystagmus, and limb ataxia in MS (single cases). In the recommended dosages, they are well tolerated. The assumed mode of action is a blockade of mainly Kv1.5: in DBN, this increases the excitability of Purkinje cells (PC), and in EA2, restores the precision of resting discharge of PC. In MS, 4-AP improves the conduction of action potentials in demyelinated axons, and in LEMS, 3,4-DAP facilitates the transmission at the neuromuscular endplate by prolonging the action potential duration.Summary:There is sufficient evidence that APs are indicated for the symptomatic treatment of DBN, EA2, gait ataxia due to MS and cerebellar disorders, and LEMS with a reasonable risk-benefit profile.
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Sieb, Jörn Peter. "3,4-diaminopyridine Phosphate in the Treatment of Lambert-Eaton Myasthenic Syndrome." European Neurological Review 7, no. 1 (2012): 56. http://dx.doi.org/10.17925/enr.2012.07.01.56.
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3,4-diaminopyridine (3,4-DAP, amifampridine) is the leading treatment for Lambert–Eaton myasthenic syndrome (LEMS), an autoimmune disorder with impaired neuromuscular transmission, for which few effective medications are currently available. 3,4-DAP has been available as a therapy for LEMS in special treatment programmes for approximately 25 years. As an unlicensed drug, doses for oral administration are required to be compounded by local, hospital or other compounding pharmacies from the base chemical. Administering the correct dose of 3,4-DAP is critical; overdosing can increase the risk of seizures and other adverse events, while underdosing can result in a substantial loss of efficacy or even treatment failure. Two recent studies, have shown a wide variation in the 3,4-DAP content of compounded preparations (22.2–125.2 %, n=9) and (53.5–128.5 %, n=21), thereby reflecting the possibility of patients receiving dosages that might be above safety limits or even markedly below efficacy limits. This inconsistency results from the variable quality and instability of the base chemical and compounding errors. A formulation of 3,4-DAP phosphate salt has now been licensed in Europe and the US with orphan medicinal product status and appears to be as efficacious as the base in relieving the symptoms of LEMS.
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Mesolella, Massimo, Salvatore Allosso, Sarah Buono, Filippo Ricciardiello, and Gaetano Motta. "Neuroendocrine carcinoma of the larynx with Lambert-Eaton myasthenic syndrome: a rare case report and literature review." Journal of International Medical Research 49, no. 5 (May 2021): 030006052110147. http://dx.doi.org/10.1177/03000605211014784.
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This current report describes a rare clinical case of neuroendocrine carcinoma (NEC) of the larynx with associated Lambert-Eaton myasthenic paraneoplastic syndrome (LEMS). A 68-year-old male patient presented with severe dysphonia and dysphagia. He underwent a total laryngectomy and the excised lesion was extremely large. A pathological examination demonstrated ‘morphological findings of a poorly differentiated carcinoma (G3) with aspects of neuroendocrine differentiation’ (i.e. a poorly differentiated neuroendocrine carcinomas [PD-NEC]). Based on the patient’s medical history and the immunohistochemical findings, he was treated with three cycles of neoadjuvant chemotherapy (cisplatin–etoposide) and then radiotherapy with a total dose of 70 Gy. Of the 10 cases of paraneoplastic syndrome (PNS) related to laryngeal NEC reported in the literature, nine of these syndromes were of an endocrine type. Only one case of PNS associated with laryngeal cancer had a neurological manifestation, which was LEMS. To the best of our knowledge, this current case has only one similar precedent in the literature and it is the second report of an association between a PD-NEC and LEMS. Laryngeal NECs are rare lesions with different prognostic characteristics. The diagnosis should be made using an endocrinological, neurological, radiological and histological multidisciplinary approach. A radical surgical approach is recommended.
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Enderwick, Peter. "Large emerging markets (LEMs) and international strategy." International Marketing Review 26, no. 1 (February 20, 2009): 7–16. http://dx.doi.org/10.1108/02651330910933177.
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Kuße, Holger. "Phantastische Objekte. Stanisław Lems Beitrag zur Semantik1." Zeitschrift für Slawistik 53, no. 1 (April 2008): 60–81. http://dx.doi.org/10.1524/slaw.2008.0004.
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Lang, B., and A. Evoli. "SOX1 autoantibodies: Tumor markers in LEMS patients?" Neurology 70, no. 12 (March 17, 2008): 906–7. http://dx.doi.org/10.1212/01.wnl.0000305966.07676.c3.
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Kohler, Siegfried, and Andreas Meisel. "Das Lambert-Eaton-Myasthenie-Syndrom: ein Überblick." Aktuelle Neurologie 45, no. 04 (May 2018): 298–304. http://dx.doi.org/10.1055/s-0043-118410.
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ZusammenfassungDas Lambert-Eaton-Myasthenie-Syndrom (LEMS) hat eine Prävalenz von etwa 5/1 000 000 und ist damit im Vergleich zur Myasthenia gravis (MG) ca. 10 – 20-mal seltener. Obwohl LEMS viele Ähnlichkeiten zur Myasthenia gravis aufweist, gibt es wichtige Unterschiede. Klinisch besteht eine vorwiegend proximal betonte belastungsabhängige Muskelschwäche, die bis hin zu einer respiratorischen Insuffizienz führen kann und häufig mit autonomen Störungen assoziiert ist. Ursächlich dafür sind Auto-Antikörper, welche gegen spannungsabhängige Kalziumkanäle (VGCC) vom P/Q-Typ an der Präsynapse gerichtet sind. Die Diagnosesicherung beruht vor allem auf der Detektion des pathologischen anti-VGCC Antikörpers sowie dem spezifischen Nachweis eines Inkrements von mindestens 60 % in der elektrophysiologischen Untersuchung eines betroffenen Muskels. Ein Inkrement ist dabei durch eine Steigerung des in Ruhe reduzierten Muskelsummenaktionspotenzials entweder nach maximaler Willkürinnervation oder hochfrequenter (≥ 20 Hz) Stimulation definiert. In einem Drittel der Patienten ist das LEMS paraneoplastischer Ätiologie, eine umfassende Tumorsuche nach Diagnosestellung ist daher notwendig. Klinisch gibt es einige Unterschiede zwischen dem paraneoplastischen (pLEMS) und dem rein autoimmunen (aiLEMS) Lambert-Eaton-Syndrom, die Hinweise geben können für die Ätiologie. Einen Anhalt dafür liefert der DELTA-P-Score und der SOX1-Antikörperstatus. Die häufigste zugrunde liegende Tumorerkrankung ist das kleinzellige Lungenkarzinom. Die Therapie basiert zunächst auf der Unterscheidung zwischen paraneoplastischer und autoimmuner Genese. pLEMS erfordert die Therapie der Tumorerkrankung. In der Regel profitieren aiLEMS- wie pLEMS-Patienten von einer symptomatischen Therapie mittels 3,4-Diaminopyridin (3,4-DAP), ggf. ergänzend Pyridostigmin sowie einer immunsuppressiven Langzeit-Therapie. Krisenhafte Verschlechterungen werden analog zur Myasthenia gravis mit Immunglobulinen, Plasmapherese oder Immunadsorption behandelt. Basierend auf positiven Erfahrungsberichten können auch moderne immunmodulatorische Ansätze, z. B. mit therapeutischen Antikörpern wie dem anti-CD20-Antikörper Rituximab, bei therapierefraktären Verläufen sinnvoll sein. Die Langzeitprognose des autoimmunen LEMS für eine klinische Stabilisierung mit weitgehender (pharmakologischer) Remission ist unserer Erfahrung nach gut, allerdings bestehen bei etwa 75 % deutliche Einschränkungen der Lebensqualität. Die Prognose der tumorassoziierten Form wird zu einem großen Teil von der Tumorerkrankung selbst und deren Therapie bestimmt. Kurative Verläufe der Tumorerkrankung und weitgehende Remission des pLEMS sind nicht selten.
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Kolev, Mirjam, Sarah Oppliger, Simon Jung, and Simon Steiner. "Neuromuskuläre Irrwege." Praxis 102, no. 2 (January 1, 2013): 111–13. http://dx.doi.org/10.1024/1661-8157/a001174.
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Wir berichten über einen 54-jährigen Patienten mit langsam progredienter, beidseitiger proximaler Beinschwäche und ataktischem Gang. Die differenzialdiagnostischen Überlegungen und Abklärungen werden beschrieben. Es ergab sich die Diagnose eines Lambert-Eaton-Myasthenie-Syndroms (LEMS) und SIADH bei kleinzelligem Bronchialkarzinom (SCLC).
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Grand'Maison, François. "Methods of Testing Neuromuscular Transmission in the Intensive Care Unit." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 25, S1 (February 1998): S36—S39. http://dx.doi.org/10.1017/s0317167100034715.
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AbstractAll disorders of neuromuscular transmission (NMT) may cause ventilatory failure, albeit rarely. Respiratory muscle weakness is occasionally the presenting feature of myasthenia gravis (MG), the Lambert-Eaton myasthenic syndrome (LEMS), hypermagnesemia and botulism. Chronic MG, congenital myasthenic syndromes and LEMS may be acutely exacerbated by various intercurrent conditions and by drugs which interfere with NMT. Finally, in the ICU, difficulty in weaning from the ventilator may be caused by prolonged use of neuromuscular blocking agents. Electrophysiological studies of NMT disorders in the intensive care unit have rarely been reported. Nevertheless, the available data indicates that the electrodiagnosis of severe NMT disorders can be misleading. With severe NMT defects, the electrophysiological distinction between post-synaptic and pre-synaptic disorders is blurred and the differential diagnosis with myopathies may be difficult. A clinically suspected NMT disorder should therefore not be ruled out when electrodiagnosis fails to demonstrate the expected abnormalities.
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Nodera, Hiroyuki, Eric L. Logigian, and David N. Herrmann. "LEMS in the shadow of a hereditary polyneuropathy." Muscle & Nerve 27, no. 5 (April 16, 2003): 636–37. http://dx.doi.org/10.1002/mus.10340.
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