Postexercise facilitation of reflexes is not common in Lambert–Eaton myasthenic syndrome

Odabaşı, Zeki; Demirci, Muhammed; Kim, D.S.; Lee, D.K.; Ryan, Hewitt F.; Claussen, Gwen C.; Tseng, Anna; Oh, Shin J.

doi:10.1212/wnl.59.7.1085

Cited by 28 publications

(12 citation statements)

References 5 publications

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“…Postexercise facilitation (a short-term return of tendon reflexes and muscle strength to normal range after muscle contraction) is a characteristic (although not very sensitive) phenomenon, which is present in 40% of patients. 32,33 Tendon reflexes should be tested after a period of rest because postexercise facilitation phenomenon can mask the hypoactive tendon reflexes.…”

Section: Clinical Featuresmentioning

confidence: 99%

Lambert-Eaton Myasthenic Syndrome

Kesner¹,

Dimachkie

et al. 2018

Neurologic Clinics

103

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Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies. 3,4-diaminopyridine is effective symptomatic treatment of LEMS.

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Section: Clinical Featuresmentioning

confidence: 99%

Lambert-Eaton Myasthenic Syndrome

Kesner¹,

Dimachkie

et al. 2018

Neurologic Clinics

103

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“…38 TR may be preserved in mild LES. 38,39 Importantly, the presence of increased TR does not exclude LES, as it might be observed in LES patients with an associated myelopathy and/or encephalopathy. The physiological mechanisms in LES that explain the reason for the rare involvement of extraocular muscles, as well as the disparity between the severely affected TR and the relatively normal muscle strength, are nicely addressed by Meriggioli and colleagues.…”

Section: Physical Examination In Lesmentioning

confidence: 92%

Section: Clinical Approach To Lesmentioning

confidence: 99%

“…The augmentation of TR, also elicited directly after a brief voluntary contraction (up to 10 s), is a unique and very useful clinical feature for diagnosing LES, although it is not very sensitive 38 . TR may be preserved in mild LES 38 39 Importantly, the presence of increased TR does not exclude LES, as it might be observed in LES patients with an associated myelopathy and/or encephalopathy.…”

Section: Clinical Approach To Lesmentioning

confidence: 99%

See 1 more Smart Citation

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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“…This facilitation may also be seen when testing muscle strength, the strength increasing after initial voluntary contraction. However, being present in only 40 % of patients it is not a very sensitive sign (Odabasi et al 2002;Oh et al 2005). This facilitation can mask the reduced tendon reflexes.…”

Section: Symptomsmentioning

confidence: 99%