@article{0c23ad0ee7a7478b8e1d2d9eb64110e9,
title = "Calcium channel autoimmunity: Cerebellar ataxia and lambert-eaton syndrome coexisting",
abstract = "Introduction: Neuronal calcium channel antibodies are a biomarker of Lambert-Eaton syndrome (LES) and cerebellar ataxia. We have encountered several patients with LES and cerebellar ataxia coexisting, and we sought to further define this association. Methods: We reviewed records of patients at our institution with a diagnosis of “Lambert-Eaton syndrome” and “cerebellar ataxia.” Results: Seventeen patients were identified with LES and ataxia. Presenting symptoms were weakness (8), concurrent weakness and ataxia (4), ataxia (4), and other (1). Nine patients had small-cell lung carcinoma, 3 of whom had survival greater than 100 months. Immunotherapy responses were best among patients without cancer. Nine of 17 (53%) patients were alive at last follow-up (median survival 62 months; range, 8–240). Discussion: Calcium channel autoimmunity should be considered in patients with coexisting cerebellar ataxia and myasthenic weakness. Affected patients may survive small-cell carcinoma or have immunotherapy-responsive neurological symptoms. Muscle Nerve 58: 29–35, 2018.",
keywords = "Lambert-Eaton syndrome, ataxia, calcium channel, myasthenic syndrome, paraneoplastic, small-cell lung carcinoma",
author = "Nicholas Zalewski and Lennon, {Vanda A.} and Pittock, {Sean J.} and Andrew Mckeon",
note = "Funding Information: Abbreviations: ANNA, antineuronal nuclear antibody; CRMP, collapsin response-mediator protein; CSF, cerebrospinal fluid; CT, computerized tomography; DELTA-P, Dutch-English LEMS Tumor Association Prediction; EMG, electromyography; IgG, immune globulin; IQR, interquartile range; LES, Lambert-Eaton syndrome; SCLC, small-cell lung carcinoma; VGCC, voltage-gated calcium channel Key words: ataxia; calcium channel; Lambert-Eaton syndrome; myasthenic syndrome; paraneoplastic; small-cell lung carcinoma Conflicts of Interest: V. A. Lennon is named inventor on a patent relating to AQP4 as NMO antigen and a pending patent related to AQP4 and cancer. Earnings to date from licensing this technology have exceeded the federal threshold for significant interest. S. J. Pittock has received no royalties to date but may accrue revenue for patents relating to AQP4 antibodies for diagnosis of neuromyelitis optica and AQP4 autoantibody as a cancer marker. He receives research support from the Guthy-Jackson Charitable Foundation, Alexion Pharmaceuticals, Inc. and the National Institutes of Health (RO1 NS065829). A. McKeon receives research support from Medimmune, Euroimmun and Grifols, and has pending patents related to GFAP, neuronal intermediate filament, and MAP1B antibodies as markers of neurological autoimmunity and cancer. N. Z. reports no disclosures. Publisher Copyright: {\textcopyright} 2017 Wiley Periodicals, Inc.",
year = "2018",
month = jul,
doi = "10.1002/mus.26053",
language = "English (US)",
volume = "58",
pages = "29--35",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "1",
}