Antigenic Sites of the Voltage‐gated Calcium Channel in Lambert‐Eaton Myasthenic Syndrome<sup>a</sup>

Takamori, Masaharu; Iwasa, Kazuo; Komai, Koichiro

doi:10.1111/j.1749-6632.1998.tb10994.x

Cited by 18 publications

(5 citation statements)

References 35 publications

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“…Numerous non‐endocrine autoimmune conditions have been reported to occur with autoimmune thyroid disease (ATD) [Torrelo et al, 1992; Puavilai et al, 1994; Coll et al, 1997; Hofbauer et al, 1997; Inoue et al, 1999]. Lambert–Eaton Myasthenic syndrome (LEMS) is a rare autoimmune condition frequently resulting from IgG autoantibodies against the P/Q voltage‐gated calcium channels (VGCC) [Takamori et al, 1998] which are located in the terminal endings of motor axons. These antibodies are pathogenic, and block the quantal release of acetylcholine presynaptically, resulting in defective neuromuscular transmission, muscle weakness, and a characteristic electrophysiologic pattern [Elmquist and Lambert, 1968].…”

Section: Introductionmentioning

confidence: 99%

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

Hoffman

Helman

Sekul

et al. 2003

American J of Med Genetics Pt A

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We report on a child with a family history of autoimmune defects, who presented at the age of 3(1/2) years with alopecia and Graves disease. He subsequently developed vitiligo and psoriasis. At 9(1/2) years, he developed an autoimmune form of Lambert-Eaton Myasthenic syndrome (LEMS) with a significant elevation of glutamic acid decarboxylase (GAD) autoantibodies. Shortly thereafter he developed chronic urticaria. HLA associations were present for Graves disease, vitiligo, psoriasis, and IgA deficiency. There was also evidence of autoimmunity involving the pancreatic islet cells and gastric parietal cells.

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Section: Introductionmentioning

confidence: 99%

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

Hoffman

Helman

Sekul

et al. 2003

American J of Med Genetics Pt A

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“…This was shown in a study in which the effect of LEMS serum on calcium influx in human embryonic kidney cells transfected with different cloned a 1 -subunits was recorded, the cells expressing the x 1A -subunit being the most effectively blocked ). In part of LEMS patients antibodies to the S5-6 extracellular region of the four domains that form the a 1A -subunit were found (Takamori et al 1998;Parsons et al 2002). Antibody response to domain I and III was similar in LEMS without tumour and paraneoplastic LEMS, but response to domain IV was more common in LEMS without tumour (Pellkofer et al 2008).…”

Section: Vgcc and Other Antigensmentioning

confidence: 93%

The Lambert-Eaton Myasthenic Syndrome

Wirtz

Titulaer

Verschuuren

2013

Pathologies of Calcium Channels

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“…57,58 These basic studies also support the hypothesis that P/Q-type VGCC antibodies are pathogenic autoantibodies in LEMS. On the other hand, although animal models of immunization due to proteins such as VGCC and synaptotagmin using peptides and recombinants have been reported, 59,60 no animal LEMS models have been successfully immunized with purified P/Qtype VGCC proteins, so the hypothesis that P/Q-type VGCC antibodies are pathogenic autoantibodies of LEMS remains inconclusive.…”

Section: Electromyographymentioning

confidence: 99%

Autoimmunity to voltage‐gated calcium channels

Motomura

Yoshimura

Shiraishi³

2023

Neurology & Clinical Neurosc

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Several review articles on voltage‐gated calcium channels (VGCCs) classify VGCCs into five electrophysiological types and 10 genotypes. Each VGCC is distributed in different organs and specialized cells, and various organ‐specific human genetic diseases have been reported. Most of the human diseases caused by abnormalities in the VGCCs are genetic diseases. To the best of our knowledge, Lambert–Eaton myasthenic syndrome (LEMS) appears to be the only autoimmune disease for VGCCs. Approximately 90% of LEMS patients are positive for autoantibodies against P/Q‐VGCCs in the active zone of nerve endings. On the other hand, about 50% of LEMS patients a tumor‐associated neurological syndrome with small cell lung cancer. Neurological symptoms in LEMS patients include weakness of proximal limb muscles, reduced tendon reflexes, autonomic neuropathy, and in 9%–14% of patients, cerebellar ataxia, and paraneoplastic cerebellar degeneration. In the second half of this manuscript, we provide a clinical review with reference to the recently published Practical Guideline for Myasthenia gravis and LEMS 2022 in Japan.

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Antigenic Sites of the Voltage‐gated Calcium Channel in Lambert‐Eaton Myasthenic Syndrome^a

Cited by 18 publications

References 35 publications

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

The Lambert-Eaton Myasthenic Syndrome

Autoimmunity to voltage‐gated calcium channels

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Antigenic Sites of the Voltage‐gated Calcium Channel in Lambert‐Eaton Myasthenic Syndromea

Cited by 18 publications

References 35 publications

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

Lambert–Eaton Myasthenic syndrome in a child with an autoimmune phenotype

The Lambert-Eaton Myasthenic Syndrome

Autoimmunity to voltage‐gated calcium channels

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Product

Resources

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Antigenic Sites of the Voltage‐gated Calcium Channel in Lambert‐Eaton Myasthenic Syndrome^a