Ocular LEMS or MLOS

Rattananan, Watcharasam; Alsharabati, Mohammad; Oh, Shin J.

doi:10.1002/mus.25189

Cited by 7 publications

(8 citation statements)

References 8 publications

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“…The diagnostic criteria for LEMS were: proximal muscle weakness and/or ocular muscle weakness; absent or decreased reflexes; and at least 60% increment after 10-second exercise or at HRS in the RNS test at the time of diagnosis. 1,4 RNS testing of the ulnar nerve was performed, recording over the abductor digiti minimi (ADM) muscle, following the Lambert method with stimulating bar electrodes at the wrist. 2,4 Disposable surface recording electrodes were placed on the skin over the ADM muscle using the belly-tendon method.…”

Section: Methodsmentioning

confidence: 99%

“…1,4 RNS testing of the ulnar nerve was performed, recording over the abductor digiti minimi (ADM) muscle, following the Lambert method with stimulating bar electrodes at the wrist. 2,4 Disposable surface recording electrodes were placed on the skin over the ADM muscle using the belly-tendon method. The hand was immobilized to minimize any movement artifact.…”

Section: Methodsmentioning

confidence: 99%

“…All patients with LEMS who participated in this study provided signed informed consent and the study was approved by our institutional review board (F9362300). The diagnostic criteria for LEMS were: proximal muscle weakness and/or ocular muscle weakness; absent or decreased reflexes; and at least 60% increment after 10‐second exercise or at HRS in the RNS test at the time of diagnosis 1,4 …”

Section: Methodsmentioning

confidence: 99%

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Assessment of the compound muscle action potential amplitude return time between exercises or tests in the repetitive nerve stimulation test for Lambert‐Eaton myasthenic syndrome

2020

Muscle and Nerve

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Introduction: When performing postexercise facilitation (PEF) as part of the repetitive nerve stimulation (RNS) test in Lambert-Eaton myasthenic syndrome (LEMS), it is important to avoid any influence of the previous exercise or RNS test on the compound muscle action potential (CMAP) amplitude. Methods: To measure the CMAP amplitude return time (ART) to that at rest, a single CMAP was obtained every 30 seconds until the amplitude was within 5% of that at rest in three exercise periods (10, 20, and 30 seconds) and in 10-second postexercise (PE) 3-Hz RNS testing with 17 tests in 10 LEMS patients. Results: Adequate ART between tests is 150 seconds for 10-second exercise (Ex) and 10-second PE 3-Hz RNS test, 120 seconds for 20-second Ex, and 90 seconds for 30-second Ex. Discussion: We recommend 150 seconds as adequate ART between the PEF test and the next test when performing RNS test in LEMS.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Assessment of the compound muscle action potential amplitude return time between exercises or tests in the repetitive nerve stimulation test for Lambert‐Eaton myasthenic syndrome

2020

Muscle and Nerve

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“…Up to 2016, 39 cases were identified as MLOS cases which met the diagnostic criteria of MOLS. Since then, additional 16 cases of MLOS were reported [30][31][32][33][34][35][36][37]. In this chapter, MOLS was discussed on the basis of 55 cases (Table 3).…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Neuromuscular junction disorders beyond myasthenia gravis

Oh¹

2021

Current Opinion in Neurology

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Purpose of reviewTo give an overview of the recent data on three autoimmune neuromuscular junction disorders with the recent Food Drug Administration (FDA) approval of amifampridine [3,4-Diaminopyridine (3,4-DAP) and 3,4-diaminopyridine phosphate (3,4-DAPP) for the treatment of Lambert-Eaton myasthenic syndrome (LEMS).Recent findingsIn LEMS, the most important recent development is the introduction of FDA approved amifampridine for the symptomatic treatment. Randomized controlled studies showed an extremely effective improvement with amifampridine with daily dose of ≤ 80 mg with minimal side reactions. The next important development is in the electrodiagnostic criteria. Now 10 s exercise and an incremental response ≥ 60% either after 10 s exercise or at the high-rate stimulation in the repetitive nerve stimulation test are recommended as the standard tests.In 2016, myasthenia-gravis Lambert-Eaton overlap syndrome (MLOS) was coined as new syndrome for patients with myasthenia gravis and LEMS combined symptoms in same patients.In Isaacs syndrome, voltage gated calcium channel antibody order is no longer recommended because of low specificity for immunotherapy responsive disorders. Instead, ‘ leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated like-2 (CASPR2) autoantibody tests’ are recommended.SummaryIn LEMS, amifampridine (3,4 DAP and 3,4-DAPP) is approved by the FDA as an effective symptomatic treatment. MLOS is coined as new syndrome recently. In Isaacs syndrome, LGI1 and CASPR2 antibody tests are recommended.

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“…In addition, cerebellar ataxia occurs in <10% of patients. Usually, the symptoms are not confined only to the extraocular muscles, as in MG . In LEMS patients, classification of severity, such as the QMG score in MG, has not been reported.…”

Section: Symptoms Classification and Prognosismentioning

confidence: 99%

Lambert–Eaton myasthenic syndrome: Clinical review

Motomura

Nakata

Shiraishi

2016

Clinical & Exp Neuroim

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Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction and approximately 60% of LEMS patients have a tumor, mostly small cell lung cancer, as a paraneoplastic neurological syndrome. LEMS patients develop a unique set of clinical characteristics, which include proximal muscle weakness, depressed tendon reflexes with posttetanic potentiation and autonomic symptoms. Interestingly, slightly <10% of LEMS patients have cerebellar ataxia (LEMS with paraneoplastic cerebellar degeneration). Considering its pathomechanism, LEMS is a presynaptic disorder of neuromuscular transmission in which quantal release of acetylcholine is impaired by autoantibodies for P/Q-type voltage-gated calcium channels at active zones, although an animal model by immunizing purified P/Q-type voltage-gated calcium channels has not yet been successful.

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Ocular LEMS or MLOS

Cited by 7 publications

References 8 publications

Assessment of the compound muscle action potential amplitude return time between exercises or tests in the repetitive nerve stimulation test for Lambert‐Eaton myasthenic syndrome

Assessment of the compound muscle action potential amplitude return time between exercises or tests in the repetitive nerve stimulation test for Lambert‐Eaton myasthenic syndrome

Neuromuscular junction disorders beyond myasthenia gravis

Lambert–Eaton myasthenic syndrome: Clinical review

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