Lambert-Eaton myasthenic syndrome: report of two cases

Scola, Rosana Hermínia; Iwamoto, Fábio M.; Ramos, Cássio S.; Sanderson, Ângela M.; Lopardo, Diego; Cantarelli, A.; Watanabe, Marcelo; Horta, Walter; Werneck, Lineü Cesar

doi:10.1590/s0004-282x1998000300017

Cited by 4 publications

(9 citation statements)

References 34 publications

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“…No treatment response was reported after intravenous immunoglobulin (4/4) and plasma exchange (2/2) ( Table 1). DISCUSSION NP-LEMS has been rarely described in Brazil, including our previous reports (cases 1, 6, 7, 8, and 9), in contrast to other places 3,12,13 . NP-LEMS usually occurs in 30 to 50% of LEMS patients 6,8 .…”

Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 64%

“…LEMS almost invariably starts with a proximal weakness (Table 2), especially of the legs 7,14 . Hyporeflexia is also a common sign (Table 2), which shows a functional improvement after a physical effort by the facilitation phenomenon that occurs by Ca +2 accumulations on the nervous terminal and, therefore, acetylcholine release on the synaptic gap 3 . Ocular symptoms are regularly seen in pa- Non-paraneoplastic Lambert-Eaton syndrome Lorenzoni et al…”

Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 99%

“…Our patients had a low frequency of the anti-P/Q-type VGCC antibody (Table 2); therefore, the LEMS patients without the anti-P/Q-type VGCC antibody more frequently have NP-LEMS than P-LEMS 6 . The classic electrophysiological abnormalities (a low CMAP amplitude, a CMAP decrease at low rate of stimulation, and a CMAP increase above 100% after a brief maximal voluntary contraction or high-frequency RNS) are present in almost all of the NP-LEMS patients, similar to P-LEMS, although this is not seen in all muscles, and it may be necessary to examine several muscles to demonstrate this feature [1][2][3]10,13 . The NP-LEMS treatment consists of symptomatic treatment of the acetylcholine deficiency as well as an immunosuppressive treatment 3,[15][16][17] .…”

Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 99%

“…Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the neuromuscular transmission in which serum autoantibodies against the P/Qtype voltage-gated calcium channels (VGCCs) at the presynaptic nerve terminal lead to a decrease in the presynaptic release of acetycholine [1][2][3] . These antibodies impair the acetylcholine release on the neuromuscular and on the autonomic neuroeffective junctions and cause the onset of the symptoms 3 .…”

Section: Introductionmentioning

confidence: 99%

“…These antibodies impair the acetylcholine release on the neuromuscular and on the autonomic neuroeffective junctions and cause the onset of the symptoms 3 . LEMS can frequently occur as remote effect of a neoplasm, in the paraneoplastic form (P-LEMS), usually in association with small cell lung cancer (SCLC), or in the nonparaneoplastic form (NP-LEMS) 1,2,[4][5][6][7][8] .…”

Section: Introductionmentioning

confidence: 99%

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Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Lorenzoni

Scola

Kay

et al. 2010

Arq. Neuro-Psiquiatr.

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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. Key words: Lambert-Eaton myasthenic syndrome, myasthenic syndrome, P/Q-type voltagegated calcium channel antibody, repetitive nerve stimulation, electrophysiological study, treatment.Síndrome miastênica de Lambert-Eaton não paraneoplásica: uma breve revisão de dez casos RESUMO A síndrome miastênica de Lambert-Eaton (LEMS) é uma desordem imunomediada da transmissão neuromuscular pré-sinaptica, que mais frequentemente ocorre como efeito à distância de uma neoplasia, na forma paraneoplásica (P-LEMS), ou na forma não paraneoplásica (NP-LEMS); porém poucos estudos têm descrito as características da NP-LEMS. Nós analisamos as manifestações clínicas, laboratoriais, eletrofisiológicas, e resposta ao tratamento em dez pacientes brasileiros com NP-LEMS. A idade média foi de 41,5 anos. A manifestação neurológica mais freqüente foi hiporeflexia ou arreflexia com melhora após o exercício. A resposta ao tratamento ocorreu com piridostigmina, guanidina, prednisona, azatioprina, e ciclosporina; mas não com imunoglobulina intravenosa e plasmaférese. A idade de início, manifestações clínicas e eletrofisiológicas ajudaram mais no diagnóstico do que os anticorpos séricos; o tratamento sintomático com piridostigmina foi efetivo; e o tratamento imunossupressor com prednisona, azatioprina, ou ciclosporina beneficiou mais do que a plasmaférese ou a imunoglobulina intravenosa. Palavras-chave: síndrome miastênica de Lambert-Eaton, síndrome miastênica, anticorpo anti-canal de cálcio voltagem dependente, estimulação nervosa repetitiva, estudo eletrofisiológico, tratamento.

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Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 64%

Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 99%

Section: Non-paraneoplastic Lambert-eaton Syndromementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Lorenzoni

Scola

Kay

et al. 2010

Arq. Neuro-Psiquiatr.

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Review of the Diagnostic Challenges of Lambert–Eaton Syndrome Revealed Through Three Case Reports

Merino-Ramirez

2016

Can. J. Neurol. Sci.

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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.RÉSUMÉ: Revue portant sur les problèmes diagnostiques du syndrome de Lambert-Eaton illustrés au moyen de trois observations cliniques. Le syndrome de Lambert-Eaton (SLE) est une maladie rare, d'origine immunitaire, caractérisée par une faiblesse proximale des jambes, des symptômes neurovégétatifs et une diminution de l'amplitude des réflexes ostéo-tendineux. La forme paranéoplasique est associée au cancer du poumon à petites cellules dans 50 à 60% des cas et les autres cas sont diagnostiqués chez des adultes plus jeunes et comportent une probabilité plus élevée de maladie autoimmune coexistante. L'identification du SLE est cruciale afin d'améliorer l'issue clinique, mais son diagnostic demeure un défi majeur. Dans cette revue, nous analysons les caractéristiques cliniques et les enjeux diagnostiques du traitement du SLE au moyen de trois observations cliniques, dont celle d'un patient qui a présenté une réponse définitive au traitement combiné par la pyridostigmine et les corticostéroïdes, suivi d'une rémission spontanée. Le dépistage chez les patients a été fait par imagerie, tests sérologiques et évaluation électrophysiologique, ainsi que par des tests respiratoires et des tests de la fonction neuro-végétative. Une meilleure compréhension des embûches fréquemment rencontrées lors du diagnostic clinique, sérologique et neurophysiologique du SLE au moment de l'évaluation de la dysfonction typique du SLE dans tout le système nerveux devrait améliorer l'identification et le traitement de ce syndrome.

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Lambert–Eaton Myasthenic Syndrome and Dermatomyositis With Anti–TIF1-gamma Autoantibody: A Unique Association of Autoimmune Neuromuscular Conditions Without Malignancy

Isfort

Mnatsakanova

Oddis

et al. 2021

Journal of Clinical Neuromuscular Disease

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Lambert–Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular junction disorder, and dermatomyositis (DM) is an idiopathic inflammatory myopathy. LEMS and DM are uncommon conditions that can present similarly and are often associated with autoantibodies. Concomitant LEMS and DM have only been reported a few times, and most of those cases were paraneoplastic. We present the first reported case of a patient with antivoltage gated calcium channel antibody positive LEMS who subsequently developed DM with antitranscription intermediary factor 1-gamma (anti–TIF1-γ) antibodies. Interestingly, both conditions occurred without evidence of malignancy. This diagnosis of LEMS and DM with characteristic clinical, electrodiagnostic, and histopathological evidence led to a beneficial modification of the patient's therapeutic regimen. Due to the fact that overlapping concurrent neuromuscular conditions are rare, a high clinical suspicion is needed to identify, evaluate (including appropriate cancer screenings), and appropriately treat these patients.

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Lambert-Eaton myasthenic syndrome: report of two cases

Cited by 4 publications

References 34 publications

Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Review of the Diagnostic Challenges of Lambert–Eaton Syndrome Revealed Through Three Case Reports

Lambert–Eaton Myasthenic Syndrome and Dermatomyositis With Anti–TIF1-gamma Autoantibody: A Unique Association of Autoimmune Neuromuscular Conditions Without Malignancy

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