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[PMID]:28930822
[Au] Autor:Portaro S; Brizzi T; Sinicropi S; Cacciola A; De Cola MC; Bramanti A; Milardi D; Lupica A; Bramanti P; Toscano A; Rodolico C
[Ad] Endereço:aIRCSS Centro Neurolesi "Bonino-Pulejo", Neuromuscular Disease Laboratory bDepartment of Clinical and Experimental Medicine, University of Messina, Messina cDIBIMIS, University of Palermo, Palermo, Italy.
[Ti] Título:Five years experience on 3,4-diaminopyridine phosphate in Lambert-Eaton syndrome: Case reports.
[So] Source:Medicine (Baltimore);96(38):e7839, 2017 Sep.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: To report our experience on 7 patients (4 males and 3 females), affected by nonparaneoplastic Lambert-Eaton myasthenic syndrome, treated with 3,4-diaminopyridine phosphate (3,4-DAPP) either alone or in combination with other immunosuppressants or steroids. PATIENT CONCERNS: Patients have been evaluated at specific timepoints (ie, baseline and last 5 year follow-up), with neurological examination, autoantibodies against presynaptic voltage-gated Cav2.1 (P/Q type) calcium ion channel (VGCC) dosage, neurophysiological evaluation focusing on the increased amplitude of the compound muscle action potential (cMAP) after maximum voluntary effort, quantitative myasthenia gravis (QMG) and activities of daily living scales, and autonomic nervous system involvement evaluation. OUTCOMES: Five out of 7 patients presented a clinical improvement persisting at last 5-year follow-up; 2 out of them improved taking only 3,4-DAPP at the maximal dosage, whereas the remaining received concomitant medications, such as prednisone and azathioprine. However, the clinical amelioration was not statistically significant. No one of the patients reported severe adverse events, except one, complaining of transient chin and perioral paresthesias. A significant association between QMG and the type of pharmacological drugs therapy (P = .028) emerged. Indeed, we observed an improvement of the clinical condition in all 3 subjects treated with 3,4-DAPP and prednisone. CONCLUSIONS: In this study, we confirm 3,4-DAPP treatment efficacy on muscle strength, but minor evidence of drug effectiveness have been demonstrated on the autonomic nervous system involvement and on the deep tendon reflexes reappearance, a part from patients who received 3,4-DAPP associated to prednisone.
[Mh] Termos MeSH primário: 4-Aminopiridina/análogos & derivados
Imunossupressores/administração & dosagem
Síndrome Miastênica de Lambert-Eaton/tratamento farmacológico
Prednisona/administração & dosagem
[Mh] Termos MeSH secundário: 4-Aminopiridina/administração & dosagem
Atividades Cotidianas
Adulto
Azatioprina/administração & dosagem
Quimioterapia Combinada
Feminino
Seres Humanos
Masculino
Meia-Idade
Força Muscular/efeitos dos fármacos
Índice de Gravidade de Doença
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunosuppressive Agents); BH3B64OKL9 (4-Aminopyridine); MRK240IY2L (Azathioprine); RU4S6E2G0J (3,4-diaminopyridine); VB0R961HZT (Prednisone)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171015
[Lr] Data última revisão:
171015
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170921
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007839


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[PMID]:28562525
[Au] Autor:Tang Y; Wang K; Chen Z; Zhou M; Duan J; Liu T; Zhou D
[Ad] Endereço:aDepartment of Neurology, West China Hospital, Sichuan University, Chengdu bDepartment of Neurology cDepartment of Ophthalmology, Mianyang Central Hospital, Mianyang, Sichuan dDepartment of Oncology, The Affiliated Jiangyin Hospital of Southeast University Medical College, Wuxi, Jiangsu, P.R. China.
[Ti] Título:Ophthalmoplegia associated with lung adenocarcinoma in a patient with the Lambert-Eaton myasthenic syndrome: A case report.
[So] Source:Medicine (Baltimore);96(22):e6484, 2017 Jun.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: The Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disease; its unique symptoms of LEMS include dry mouth with a metallic taste, constipation, and erectile dysfunction. As it is quite rare, isolated ocular muscle impairment associated with LEMS east to ignore. PATIENT CONCERNS: A 65-year-old man presented with alternating ptosis and diplopia. Isolated ocular muscle impairment had lasted for 6 years, and the patient was initially diagnosed with ocular myasthenia gravis (MG). Treatment with azathioprine only slightly improved symptoms over the first 2 months; long-term treatment was not effective. DIAGNOSES: Dynamic observation of chest computed tomography images revealed a slowly progressing nodule in the lower lobe of the left lung. The subsequent pathologic examination following mass resection confirmed a diagnosis of lung adenocarcinoma. INTERVENTIONS: The patient was ultimately diagnosed with the Lambert-Eaton myasthenic syndrome associated with pulmonary adenocarcinoma. OUTCOMES: Resection of the lung tumor relieved all symptoms. LESSONS: Other causes of ocular MG symptoms should be considered when standard MG therapy is ineffective, especially the Lambert-Eaton myasthenic syndrome.
[Mh] Termos MeSH primário: Adenocarcinoma/complicações
Síndrome Miastênica de Lambert-Eaton/complicações
Neoplasias Pulmonares/complicações
Oftalmoplegia/etiologia
[Mh] Termos MeSH secundário: Adenocarcinoma/diagnóstico por imagem
Adenocarcinoma/patologia
Adenocarcinoma/cirurgia
Idoso
Diagnóstico Diferencial
Seres Humanos
Síndrome Miastênica de Lambert-Eaton/diagnóstico por imagem
Síndrome Miastênica de Lambert-Eaton/tratamento farmacológico
Síndrome Miastênica de Lambert-Eaton/cirurgia
Neoplasias Pulmonares/diagnóstico por imagem
Neoplasias Pulmonares/patologia
Neoplasias Pulmonares/cirurgia
Masculino
Oftalmoplegia/diagnóstico por imagem
Oftalmoplegia/tratamento farmacológico
Oftalmoplegia/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170626
[Lr] Data última revisão:
170626
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170601
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000006484


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[PMID]:28374700
[Au] Autor:Tumurov DA; Sanadze AG
[Ad] Endereço:Moscow City Health Department, Moscow Myasthenia Center, SFHI Municipal Clinical Hospital No. 51, Moscow, Russia; Moscow Research and Clinical Center for Neuropsychiatry, Moscow, Russia.
[Ti] Título:[Decrement pattern of M-response amplitude in the low-frequency repetitive nerve stimulation in the muscles of patients with myasthenia gravis and Lambert-Eaton myasthenic syndrome].
[Ti] Título:Dekrement amplitudy M-otveta pri nizkochastotnoi stimulyatsii myshts bol'nykh miasteniei i miastenicheskim sindromom Lamberta­Itona..
[So] Source:Zh Nevrol Psikhiatr Im S S Korsakova;117(2):93-96, 2017.
[Is] ISSN:1997-7298
[Cp] País de publicação:Russia (Federation)
[La] Idioma:rus
[Ab] Resumo:AIM: To investigate the pattern of decrement in the muscles of patients with myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). MATERIAL AND METHODS: Twenty-seven patients with MG and 39 patients with LEMS were studied using low frequency repetitive nerve stimulation (3/ sec). RESULTS AND CONCLUSION: The decrease of safety factor of neuromuscular transmission was equal in both groups. At the same time, a significant difference in the decrease of pattern of the amplitude compound of muscle action potential (CMAP) was found. In LEMS, by contrast with MG, another sequence of amplitude variability of CMAP from the second stimulus to the first and from the fifth stimulus to the fourth was noted. In LEMS patients, progressive decrement, manifesting by increasing ratios of late A9/A1 to early A4/A1 was found, whereas the MG patients showed retrogressive decrement expressed by the reduction in decrement ratio (from late to early). These differences reflect the mechanisms and status of acetylcholine mobilization and release from the axon terminal.
[Mh] Termos MeSH primário: Síndrome Miastênica de Lambert-Eaton/fisiopatologia
Miastenia Gravis/fisiopatologia
Transmissão Sináptica
[Mh] Termos MeSH secundário: Acetilcolina/metabolismo
Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Axônios/metabolismo
Eletromiografia
Seres Humanos
Meia-Idade
Exame Neurológico
Estimulação Elétrica Nervosa Transcutânea
Adulto Jovem
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Nm] Nome de substância:
N9YNS0M02X (Acetylcholine)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170810
[Lr] Data última revisão:
170810
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170405
[St] Status:MEDLINE
[do] DOI:10.17116/jnevro20171172193-96


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[PMID]:28251917
[Au] Autor:Maddison P; Gozzard P; Grainge MJ; Lang B
[Ad] Endereço:From the Department of Neurology (P.M.), Nottingham University Hospitals NHS Trust, Queen's Medical Centre; Division of Clinical Neuroscience (P.G.), University of Nottingham, Queen's Medical Centre; Department of Statistics (M.J.G.), University of Nottingham, Nottingham City Hospital; and Nuffield
[Ti] Título:Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome.
[So] Source:Neurology;88(14):1334-1339, 2017 Apr 04.
[Is] ISSN:1526-632X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo: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, = 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.
[Mh] Termos MeSH primário: Síndrome Miastênica de Lambert-Eaton/complicações
Síndrome Miastênica de Lambert-Eaton/mortalidade
Carcinoma de Pequenas Células do Pulmão/complicações
Carcinoma de Pequenas Células do Pulmão/mortalidade
[Mh] Termos MeSH secundário: Adulto
Fatores Etários
Autoanticorpos/análise
Estudos de Coortes
Feminino
Seres Humanos
Masculino
Prognóstico
Modelos de Riscos Proporcionais
Fatores Sexuais
Sódio/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Autoantibodies); 9NEZ333N27 (Sodium)
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170523
[Lr] Data última revisão:
170523
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170303
[St] Status:MEDLINE
[do] DOI:10.1212/WNL.0000000000003794


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[PMID]:28178777
[Au] Autor:Avina Fierro JA; Hernandez Avina DA
[Ad] Endereço:Department of Pediatric Dysmorphology, Mexican Social Security Institute (IMSS) Medical Center, Guadalajara, Mexico - avinafie@megared.net.mx.
[Ti] Título:Pediatric Lambert-Eaton Myasthenic Syndrome.
[So] Source:Minerva Pediatr;69(2):161-162, 2017 Apr.
[Is] ISSN:1827-1715
[Cp] País de publicação:Italy
[La] Idioma:eng
[Mh] Termos MeSH primário: Blefaroptose/etiologia
Síndrome Miastênica de Lambert-Eaton/terapia
[Mh] Termos MeSH secundário: Blefaroptose/terapia
Pré-Escolar
Seres Humanos
Síndrome Miastênica de Lambert-Eaton/diagnóstico
Síndrome Miastênica de Lambert-Eaton/fisiopatologia
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170210
[St] Status:MEDLINE
[do] DOI:10.23736/S0026-4946.16.04332-2


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[PMID]:28006860
[Au] Autor:Pavolucci L; Giannini G; Giannoccaro MP; Foschini MP; Lang B; Avoni P; Tinuper P; Vincent A; Liguori R
[Ad] Endereço:Department of Biomedical and Neuromotor Sciences (DiBiNeM), University of Bologna, Via Altura 3, 40139, Bologna, Italy.
[Ti] Título:Paraneoplastic cerebellar degeneration and lambert-eaton myasthenia in a patient with merkel cell carcinoma and voltage-gated calcium channel antibodies.
[So] Source:Muscle Nerve;56(5):998-1000, 2017 Nov.
[Is] ISSN:1097-4598
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. METHODS: A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Subsequently he also developed dysarthria, diplopia, xerostomia, fatigability and progressive anorexia. He underwent a full diagnostic workup and was found to have a high titer of voltage-gated calcium channel antibodies in serum and cerebrospinal fluid, neurophysiological findings compatible with Lambert-Eaton myasthenia and neurological signs compatible with cerebellar degeneration. RESULTS: A positron emission tomography study revealed a hypermetabolic lesion in the axilla, subsequently biopsied and consistent with Merkel cell carcinoma. CONCLUSIONS: In most previous reports, neurological symptoms preceded the Merkel cell carcinoma diagnosis, and the primary localization was in lymph nodes. This tumor should be considered in patients with paraneoplastic syndrome, and particularly Lambert-Eaton myasthenia after exclusion of small cell lung carcinoma. Muscle Nerve 56: 998-1000, 2017.
[Mh] Termos MeSH primário: Autoanticorpos/sangue
Canais de Cálcio Tipo N/imunologia
Carcinoma de Célula de Merkel
Síndrome Miastênica de Lambert-Eaton
Neoplasias Pulmonares
Degeneração Paraneoplásica Cerebelar
[Mh] Termos MeSH secundário: Idoso
Carcinoma de Célula de Merkel/sangue
Carcinoma de Célula de Merkel/complicações
Carcinoma de Célula de Merkel/imunologia
Seres Humanos
Neoplasias Pulmonares/sangue
Neoplasias Pulmonares/complicações
Neoplasias Pulmonares/imunologia
Masculino
Degeneração Paraneoplásica Cerebelar/sangue
Degeneração Paraneoplásica Cerebelar/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Autoantibodies); 0 (Calcium Channels, N-Type)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171019
[Lr] Data última revisão:
171019
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161223
[St] Status:MEDLINE
[do] DOI:10.1002/mus.25530


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[PMID]:27997683
[Au] Autor:Abenroth DC; Smith AG; Greenlee JE; Austin SD; Clardy SL
[Ad] Endereço:Department of Neurology, Veterans Affairs Medical Center, Salt Lake City, Utah, USA.
[Ti] Título:Lambert-Eaton myasthenic syndrome: Epidemiology and therapeutic response in the national veterans affairs population.
[So] Source:Muscle Nerve;56(3):421-426, 2017 Sep.
[Is] ISSN:1097-4598
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: One nationwide study (The Netherlands) of Lambert-Eaton myasthenic syndrome (LEMS) has been published. We report LEMS epidemiology and its therapeutic response in the United States Veterans Affairs (VA) population. METHODS: Medical records for all active patients (12.5 million) in the VA health system were queried for relevant ICD-9 codes for the period October 1, 1999 to September 30, 2013. Clinical, electrophysiologic, and serologic features were evaluated to confirm diagnosis; epidemiologic and treatment data were collected. RESULTS: Point prevalence was estimated at 2.6 per 1,000,000 (confirmed cases) and 3.3 per 1,000,000 (combined confirmed and probable cases). Crude prevalence was similarly estimated at 9.2 and 10.9 per 1,000,000 respectively. A total of 18 of 48 (38%) patients received 3,4-diaminopyridine (3,4-DAP); 14 of 18 (78%) improved. CONCLUSIONS: This investigation was a large North American epidemiologic study of LEMS. LEMS prevalence in the national VA population was found to be similar to previously published rates in other large international populations. Most patients experienced improvement with therapy, including a majority with 3,4-DAP. Muscle Nerve 56: 421-426, 2017.
[Mh] Termos MeSH primário: Síndrome Miastênica de Lambert-Eaton/epidemiologia
Síndrome Miastênica de Lambert-Eaton/terapia
Vigilância da População
United States Department of Veterans Affairs
Veteranos
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Feminino
Seres Humanos
Imunoglobulinas Intravenosas/uso terapêutico
Masculino
Meia-Idade
Plasmaferese/tendências
Vigilância da População/métodos
Bloqueadores dos Canais de Potássio/uso terapêutico
Estudos Retrospectivos
Resultado do Tratamento
Estados Unidos/epidemiologia
United States Department of Veterans Affairs/tendências
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunoglobulins, Intravenous); 0 (Potassium Channel Blockers)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170911
[Lr] Data última revisão:
170911
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161221
[St] Status:MEDLINE
[do] DOI:10.1002/mus.25520


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[PMID]:27935068
[Au] Autor:Guan Y; Ding Q; Liu M; Niu J; Cui L
[Ad] Endereço:Department of Neurology, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Wangfujing, Dongcheng, Beijing, China, 100730.
[Ti] Título:Single-fiber EMG with concentric electrodes in lambert-eaton myasthenia.
[So] Source:Muscle Nerve;56(2):253-257, 2017 Aug.
[Is] ISSN:1097-4598
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: We analyzed jitter recordings made with concentric needle electrode (CNE) single-fiber electromyography (SFEMG) in Lambert-Eaton myasthenia (LEM). METHODS: Fifteen subjects diagnosed with LEM were studied using CNE-SFEMG in the extensor digitorum (ED) and tibialis anterior (TA) muscles. CNE-SFEMG in the ED and TA was also used to evaluate 12 and 10 healthy controls (HCs), respectively. RESULTS: Ten men and 5 women were diagnosed with LEM based on an increase of 100% in compound muscle action potential amplitude during 50 Hz repetitive nerve stimulation. All patients exhibited markedly greater jitter in the ED (88.8 ± 23.2 µs) and TA (92.2 ± 30.2 µs) than HCs (28.3 ± 3.4 µs and 30.9 ± 5.1 µs, respectively). CONCLUSIONS: CNE-SFEMG is sensitive for discovering abnormalities in neuromuscular transmission in LEM. Muscle Nerve 56: 253-257, 2017.
[Mh] Termos MeSH primário: Síndrome Miastênica de Lambert-Eaton/patologia
Fibras Musculares Esqueléticas/fisiologia
[Mh] Termos MeSH secundário: Potenciais de Ação/fisiologia
Adulto
Idoso
Estimulação Elétrica
Eletrodos
Eletromiografia
Fadiga/etiologia
Feminino
Seres Humanos
Imunoglobulinas Intravenosas/uso terapêutico
Síndrome Miastênica de Lambert-Eaton/complicações
Síndrome Miastênica de Lambert-Eaton/tratamento farmacológico
Masculino
Meia-Idade
Debilidade Muscular/etiologia
Condução Nervosa/fisiologia
Reflexo/fisiologia
Estudos Retrospectivos
Estatísticas não Paramétricas
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunoglobulins, Intravenous)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170818
[Lr] Data última revisão:
170818
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161210
[St] Status:MEDLINE
[do] DOI:10.1002/mus.25500


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[PMID]:27506750
[Au] Autor:Chan KY; Chang RS; Lau VW; Chan ML; Lai T
[Ad] Endereço:Palliative Medical Unit, Grantham Hospital, 125 Wong Chuk Hang, Hong Kong, China. cky842@yahoo.com.hk.
[Ti] Título:Palliative care for a patient with Lambert-Eaton myasthenic syndrome: role of 3,4-diaminopyridine.
[So] Source:Ann Palliat Med;5(4):311-314, 2016 Oct.
[Is] ISSN:2224-5839
[Cp] País de publicação:China
[La] Idioma:eng
[Ab] Resumo:Lambert-Eaton myasthenic syndrome (LEMS) is an uncommon autoimmune idiopathic or paraneoplastic syndrome producing antibodies against voltage presynaptic calcium channels. According to previous studies, many patients with LEMS experience remission in both the clinical symptoms of muscle weakness and the electrophysiologic abnormalities after successful treatment of lung SCC. However, some patients might not respond to conventional therapy and eventually require palliative care. Hereby, we reported a LEMS patient with advanced lung malignancy was referred for palliative care. He was benefited from multidisciplinary approach even with limited survival. In this case, use of 3,4-diaminopyridine (3,4-DAP) had other roles apart from symptomatic treatment.
[Mh] Termos MeSH primário: 4-Aminopiridina/análogos & derivados
Síndrome Miastênica de Lambert-Eaton/tratamento farmacológico
Cuidados Paliativos/métodos
Bloqueadores dos Canais de Potássio/uso terapêutico
[Mh] Termos MeSH secundário: 4-Aminopiridina/uso terapêutico
Potenciais de Ação/efeitos dos fármacos
Feminino
Seres Humanos
Masculino
Meia-Idade
Condução Nervosa/efeitos dos fármacos
Doente Terminal
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Potassium Channel Blockers); BH3B64OKL9 (4-Aminopyridine); RU4S6E2G0J (3,4-diaminopyridine)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170215
[Lr] Data última revisão:
170215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160811
[St] Status:MEDLINE
[do] DOI:10.21037/apm.2016.06.01


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[PMID]:27412406
[Au] Autor:Merino-Ramírez MÁ; Bolton CF
[Ad] Endereço:1Department of Clinical Neurophysiology,Hospital Universitario de la Ribera,Alzira,Valencia,Spain.
[Ti] Título:Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports.
[So] Source:Can J Neurol Sci;43(5):635-47, 2016 Sep.
[Is] ISSN:0317-1671
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Lambert-Eaton syndrome (LES) is a rare immune-mediated disorder characterized by proximal leg weakness, autonomic symptoms and hypoactive tendon reflexes. The paraneoplastic form is associated with small-cell lung cancer in 50-60% of cases, whereas the remaining cases are found in younger adults with a higher likelihood of coexisting autoimmune disease. The early recognition of LES is crucial for improving clinical outcomes but remains a major challenge. In this review, we analyze the clinical characteristics and diagnostic considerations in treating LES through a series of three case studies, one of which showed definitive response to pyridostigmine and corticosteroid combination therapy, followed by spontaneous remission. Patients were assessed by image-based screening, serological testing and electrophysiological evaluations, which included respiratory and autonomic testing. A better understanding of the common pitfalls in the clinical, serological and neurophysiologic diagnosis of LES through assessment of typical LES dysfunction throughout the nervous system should enable improved recognition and treatment of this syndrome.
[Mh] Termos MeSH primário: Síndrome Miastênica de Lambert-Eaton/diagnóstico
Síndrome Miastênica de Lambert-Eaton/fisiopatologia
[Mh] Termos MeSH secundário: Potenciais de Ação/fisiologia
Adulto
Idoso
Estimulação Elétrica
Eletromiografia
Potencial Evocado Motor/fisiologia
Feminino
Seres Humanos
Masculino
Meia-Idade
Debilidade Muscular/etiologia
Condução Nervosa/fisiologia
Transtornos Respiratórios/diagnóstico
Transtornos Respiratórios/etiologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160715
[St] Status:MEDLINE
[do] DOI:10.1017/cjn.2016.268



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