Abstract：
:A high index of suspicion is essential in arriving at the correct diagnosis of Lambert-Eaton myasthenic syndrome (LEMS). LEMS should be considered in the differential in any patient who has proximal weakness, reduced or absent muscle stretch reflexes, and dry mouth. Weakness predominates in hip and shoulder muscles, but may also affect ocular and oropharyngeal muscles to a lesser extent. The diagnosis is confirmed by demonstrating characteristic electromyographic findings-low-amplitude muscle responses that increase dramatically after activation. Most patients also have circulating antibodies to the voltage-gated calcium channel. Half the patients with LEMS have a malignancy, usually small-cell lung cancer. The diagnosis should trigger an intensive search for malignancy, especially in older patients with a history of smoking. Younger, nonsmoking patients are likely to have LEMS as part of a more general autoimmune state. Successful treatment of the underlying cancer leads to improvement in many patients. More than 85% of patients have clinically significant benefit from 3,4-diaminopyridine (DAP). In over half of these, the improvement is marked. If severe weakness persists despite DAP, immunotherapy should be considered. Plasma exchange and high-dose immunoglobulin induce transient improvement in many patients, but function rarely becomes normal. Combinations of prednisone, azathioprine, or cyclosporine have been used with variable success. Improvement, if any, occurs only after many months and requires chronic administration of immunosuppressive medications at significant doses. The long-term prognosis in LEMS is determined by the presence of cancer or other autoimmune disease.
journal_name
Ann N Y Acad Scijournal_title
Annals of the New York Academy of Sciencesauthors
Sanders DBdoi
10.1196/annals.1254.065subject
Has Abstractpub_date
2003-09-01 00:00:00pages
500-8eissn
0077-8923issn
1749-6632journal_volume
998pub_type
杂志文章,评审abstract：:The cell surface antigen recognized by monoclonal antibody W7C5 is expressed at low levels on human CD34(+) and CD34(-) bone marrow stem cell populations but at high levels on fetal liver CD34(+) cells. To identify the recognized antigen, we performed partial amino acid sequence and MALDI analysis of purified W7C5 ant...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.2003.tb03250.x
更新日期：2003-05-01 00:00:00
abstract：:Monoclonal antibodies reactive with desmin (D3 and D76) have been generated and their specificities validated by immunoblots, RIAs, and immunocytochemistry. No cross-reaction with other IFPs has been observed. The McAbs recognized different epitopes but both reside in the amino-terminal rod domain of desmin. Whereas M...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1985.tb50411.x
更新日期：1985-01-01 00:00:00
abstract：:The adult respiratory distress syndrome (ARDS) is not confined only to late shock states but is a typical finding in intoxications with such oxidizing agents as O3, NO2, chlorate, or paraquat. Under experimental conditions, alpha-tocopherol in a high enteric dosage (2 x 50 mg/kg) was able to prevent alterations of pul...
journal_title：Annals of the New York Academy of Sciences
pub_type： 临床试验,杂志文章
doi：10.1111/j.1749-6632.1982.tb31278.x
更新日期：1982-01-01 00:00:00
abstract：:Auditory stimulation via rhythmic cues can be used successfully in the rehabilitation of motor function in patients with motor disorders. A prototypical example is provided by dysfunctional gait in patients with idiopathic Parkinson's disease (PD). Coupling steps to external rhythmic cues (the beat of music or the sou...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/nyas.12651
更新日期：2015-03-01 00:00:00
abstract：:The HLA DQB1*0302 allele on DR4 haplotypes is a well-known marker for T1D susceptibility. The contribution of this DQ molecule to overall disease susceptibility may be genotype dependent. On Asian DR4 haplotypes, not only DQB1*0302, but also DQB1*0401 confer susceptibility to T1D, which is mediated by DRB1*0405 on the...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1196/annals.1337.015
更新日期：2004-12-01 00:00:00
abstract：:Fibronectin is a ubiquitous extracellular matrix (ECM) protein known to play a critical role in cell adhesion. In the present study we dissected the effects of glucocorticoids and cyclic adenosine 3',5'-monophosphate (cAMP) on FN expression in cultures of cytotrophoblasts isolated from human term placentas to identify...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1994.tb21741.x
更新日期：1994-09-30 00:00:00
abstract：:The loss of mucus coat continuity and apoptosis have been shown in Helicobacter pylori (H. pylori)-infected gastric tissues. Blockade of p38 mitogen-activated kinase (MAPK) produced reversal in the LPS-induced reduction in mucin synthesis and apoptosis in gastric epithelial cells. This study investigates whether H. py...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1196/annals.1299.014
更新日期：2003-12-01 00:00:00
abstract：:The study of the morphological phenotypes in patients with different mutations of the PMP22 gene gives additional insights into the role of the protein in myelin function. The pathology in young patients is in some aspects different from the pathology in older patients, providing essential and additional information a...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1999.tb08595.x
更新日期：1999-10-01 00:00:00
abstract：:In the basal state, patients with sarcoidosis have reduced numbers of circulating T lymphocytes and a normal number of circulating B lymphocytes. The lymphocytes show impaired DNA synthesis in response to several mitogens and antigens and impaired K-cell cytotoxicity. Circulating atypical cells of lymphocytic origin a...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1976.tb47020.x
更新日期：1976-01-01 00:00:00
abstract：:The airway disease termed asthma has been recognized for centuries but is now receiving increased attention because of chronicity, high incidence in the population and rising mortality rate. Asthma is now recognized as a disease of unknown etiology characterized by a hyperactive airway which can be demonstrated by res...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.1991.tb37954.x
更新日期：1991-01-01 00:00:00
abstract：:After fertilization, the next major hurdle for human reproduction is trophoblast differentiation, which is required for implantation, followed in lockstep by rapid assembly of these embryonic cells into a functional placenta. During this process, cytotrophoblast stem cells invade the uterus, anchoring the conceptus to...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.2003.tb03211.x
更新日期：2003-05-01 00:00:00
abstract：:Substitution of T4 with T3 for T4 replacement in patients with hypothyroidism was undertaken using a randomized placebo controlled study design. Forty individuals were included who had depressive symptoms on stable doses of levothyroxine. Combined T4 plus T3 did not have a significantly different effect on mood and we...
journal_title：Annals of the New York Academy of Sciences
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1196/annals.1314.040
更新日期：2004-12-01 00:00:00
abstract：:Astrakhan fever is a summer spotted fever resembling Mediterranean spotted fever, endemic in Astrakhan, a region of Russia located by the Caspian sea. Its agent is a spotted fever group rickettsia, member of the Rickettsia conorii complex, transmitted to humans by Rhipicephalus sanguineus and Rhipicephalus pumilio tic...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.2003.tb07357.x
更新日期：2003-06-01 00:00:00
abstract：:Activated platelets and leukocytes release potent vasoactive factors that may modulate vascular tone. Activation of normal platelets produces dilatation of normal arteries. Vasodilatation is mediated by adenosine diphosphate (ADP), which releases endothelium-derived relaxing factor (EDRF) from endothelium. In atherosc...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.1994.tb12036.x
更新日期：1994-04-18 00:00:00
abstract：:To minimize technical problems relating to excessive size (600-800 mu in diameter) of standard alginate microcapsules (CSM) for pancreatic islet graft immunoisolation, we have developed two novel minimal volume, chemically identical, capsule prototypes (MVC): 1) coherent microcapsules (CM), and 2) medium-size microcap...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.1999.tb08506.x
更新日期：1999-06-18 00:00:00
abstract：:We have described a family of adrenocortical tumor cell mutants (including clones OS3, Y6, and 10r9) that are resistant to ACTH because they fail to express the gene encoding the ACTH receptor (MC2R). The MC2R deficiency results from a mutation that impairs the activity of the nuclear receptor steroidogenic factor 1 (...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.2003.tb03174.x
更新日期：2003-06-01 00:00:00
abstract：:Tight and adherens junctions play critical roles in the barrier, adhesion, and signaling functions of epithelial and endothelial cells. How the molecular organization of these junctions is tuned to the widely diverse physiological requirements of each tissue type is not well understood. Here, we address this question ...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/nyas.13391
更新日期：2017-10-01 00:00:00
abstract：:This paper explores aspects of the role that laboratory instruments have played in establishing psychology as a science. Comparisons of earlier instruments with their more modern counterparts indicate that many problems and methods still of interest today were explored in the laboratories of psychology's pioneers. Suc...
journal_title：Annals of the New York Academy of Sciences
pub_type： 历史文章,杂志文章
doi：10.1111/j.1749-6632.1983.tb47336.x
更新日期：1983-01-01 00:00:00
abstract：:In Bar Harbor 129 dystrophic mice, thymic development is abnormal. Before weaning, the thymus is slightly smaller than in phenotypically normal littermates; after weaning, however, the lymphoid elements undergo rapid atrophy. The epithelial elements, in contrast, display hyperlasia. Thymectomy has no influence on the ...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1979.tb56574.x
更新日期：1979-01-01 00:00:00
abstract：:The Ras proteins, which regulate intracellular signaling by a cyclic process involving interconversion between active GTP-bound and inactive GDP-bound states, play an essential role in controlling the activity of several crucial signaling pathways regulating normal cellular proliferation. Mutational activation of RAS ...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1196/annals.1329.007
更新日期：2004-12-01 00:00:00
abstract：:On occasion, emerging scientific fields intersect and great discoveries result. In the last decade, the discovery of regulatory T cells (T(reg)) in immunity has revolutionized our understanding of how the immune system is controlled. Intersecting the rapidly emerging field of T(reg) function, has been the discovery th...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1196/annals.1443.017
更新日期：2008-11-01 00:00:00
abstract：:Our studies of Ca2+ release have shown that three classes of compounds--quercetin, which inhibits Ca2+ release by reversal of the Ca2+ pump, H+ ionophores and DCCD, which affect proton gradients, and lipophilic anions, which act to oppose a separation of membrane surface charges--all have potential in probing the mech...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1982.tb25769.x
更新日期：1982-01-01 00:00:00
abstract：:Stationary-phase mutation (a subset of which was previously called adaptive mutation) occurs in apparently nondividing, stationary-phase cells exposed to a nonlethal genetic selection. In one experimental system, stationary-phase reversion of an Escherichia coli F'-borne lac frameshift mutation occurs by a novel molec...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.1999.tb08888.x
更新日期：1999-05-18 00:00:00
abstract：:Unfortunately, all of the problems of the hemophiliac have not been solved by the availability of concentrated factor VIII products. Patients still are faced with the crippling effects of arthritis, problems with employment, problems with ignorance (both medical and lay), and an increased risk of premature death even ...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1111/j.1749-6632.1975.tb53341.x
更新日期：1975-01-20 00:00:00
abstract：:The presence in the serum of specific autoantibodies, such as antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), and antiextractable nuclear antigens (anti-ENA), is one of the diagnostic criteria for autoimmune rheumatic disease, and the requests for these tests in the last few years have grown remar...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,多中心研究
doi：10.1111/j.1749-6632.2009.04635.x
更新日期：2009-09-01 00:00:00
abstract：:The effect of 310 courses of corticotropin, methylprednisolone, prednisone, and dexamethasone were studied in 62 patients with generalized myasthenia gravis who were poorly responsive to anticholinesterase medication and most of whom required assisted respiration. Improvement in strength and response to anticholinest...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.1976.tb47717.x
更新日期：1976-01-01 00:00:00
abstract：:Pituitary adenylate cyclase-activating polypeptide (PACAP) is a structurally highly conserved neuropeptide and displays pleiotropic activity, including functioning as a neurotransmitter, neuromodulator, and neurotrophic factor. A series of recent experiments, including genetic manipulation of PACAP and its receptors, ...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1196/annals.1317.038
更新日期：2006-07-01 00:00:00
abstract：:Monitoring of wildlife diseases started in Scandinavia as sporadic postmortem examinations in the early 20th century. In 1945 a monitoring program for wildlife health was initiated. The program is today an integrated part of the National Environmental Monitoring programs in Sweden. The total material today comprises m...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章
doi：10.1111/j.1749-6632.2002.tb04347.x
更新日期：2002-10-01 00:00:00
abstract：:Insulin-like factor 3 (INSL3), previously known as the relaxin-like factor (RLF), is a major peptide hormone secreted from the testicular Leydig cells of adult men and circulating in the blood at a concentration of approximately 1 ng/mL. Women also produce INSL3 in the theca interna cells of ovarian follicles, but cir...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1196/annals.1282.073
更新日期：2005-05-01 00:00:00
abstract：:Endometriosis is often a perplexing medical condition for both the physician and the patient. Accordingly, development of treatment strategies based on the needs of the individual patient is highly desirable. Although endometriosis has been part of the clinical practice for almost a century, many questions remain rela...
journal_title：Annals of the New York Academy of Sciences
pub_type： 杂志文章,评审
doi：10.1196/annals.1290.026
更新日期：2003-11-01 00:00:00
