Efficacy of intravenous immunoglobulin for treatment of Lambert–Eaton myasthenic syndrome without anti-presynaptic P/Q-type voltage-gated calcium channel antibodies: A case report

Okada, Ayaka; Koike, Haruki; Nakamura, Tomohiko; Motomura, M; Sobue, Gen

doi:10.1016/j.nmd.2014.08.006

Cited by 6 publications

(3 citation statements)

References 19 publications

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“…Oral prednisone, alone or in combination with AZA, has been shown to be effective in a retrospective study [101]. However, IVIG is suggested as first-line treatment [102] in patients with LEMS, based on case reports [103,104] and a placebo-controlled crossover trial showing significant improvement in limb strength after treatment [59]. PLEX has been reported as LEMS treatment in case reports/series but there are not clinical trials [101,105,106].…”

Section: Treatment and Prognosismentioning

confidence: 99%

Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

2021

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The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.

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Section: Treatment and Prognosismentioning

confidence: 99%

Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

2021

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“…According to clinical studies, AAN has endorsed the clinical use of IVIg as supported by evidence of efficacy in the treatment of MG (level B) and LEMS (level C). Some reports and single randomized placebo-controlled crossover studies found clinical improvement in LEMS patients after treatment with IVIg, peaking at 2–4 weeks, and declining by 8 weeks (76, 82–84). Plasma exchange (PE) has been reported in case series and case reports but are lack of clinical trials in LEMS patients.…”

Section: Emgmentioning

confidence: 99%

Autoimmune Channelopathies at Neuromuscular Junction

2019

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The neuromuscular junction, also called myoneural junction, is a site of chemical communication between a nerve fiber and a muscle cell. There are many types of channels at neuromuscular junction that play indispensable roles in neuromuscular signal transmission, such as voltage-gated calcium channels and voltage-gated potassium channels on presynaptic membrane, and acetylcholine receptors on post-synaptic membrane. Over the last two decades, our understanding of the role that autoantibodies play in neuromuscular junction disorders has been greatly improved. Antibodies against these channels cause a heterogeneous group of diseases, such as Lambert-Eaton syndrome, Isaacs' syndrome and myasthenia gravis. Lambert-Eaton syndrome is characterized by late onset of fatigue, skeletal muscle weakness, and autonomic symptoms. Patients with Isaacs' syndrome demonstrate muscle cramps and fasciculation. Myasthenia gravis is the most common autoimmune neuromuscular junction channelopathy characterized by fluctuation of muscle weakness. All these disorders have a high risk of tumor. Although these channelopathies share some common features, they differ for clinical features, antibodies profile, neurophysiological features, and treatments. The purpose of this review is to give a comprehensive insight on recent advances in autoimmune channelopathies at the neuromuscular junction.

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“…If these treatments are ineffective, steroid therapy and immunosuppressants can be used. If the immunotherapy does not sufficiently improve muscle weaknesses and residual severe motor impairment occurs, or in cases in which immunotherapy is contraindicated, plasma exchange and/or high‐dose intravenous immunoglobulin treatments are recommended. These treatments are selected with regard to treatments used for MG. Rituximab is being considered for refractory LEMS patients and its utility has been reported .…”

Section: Treatmentmentioning

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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Efficacy of intravenous immunoglobulin for treatment of Lambert–Eaton myasthenic syndrome without anti-presynaptic P/Q-type voltage-gated calcium channel antibodies: A case report

Cited by 6 publications

References 19 publications

Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

Autoimmune Channelopathies at Neuromuscular Junction

Lambert–Eaton myasthenic syndrome: Clinical review

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