Calcium channel autoimmunity: Cerebellar ataxia and lambert-eaton syndrome coexisting

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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.

Original languageEnglish (US)
JournalMuscle and Nerve
DOIs
StateAccepted/In press - Jan 1 2018

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Lambert-Eaton Myasthenic Syndrome
Cerebellar Ataxia
Calcium Channels
Autoimmunity
Ataxia
Immunotherapy
Small Cell Carcinoma
Survival
Muscle Weakness
Small Cell Lung Carcinoma
Biomarkers

Keywords

  • Ataxia
  • Calcium channel
  • Lambert-Eaton syndrome
  • Myasthenic syndrome
  • Paraneoplastic
  • Small-cell lung carcinoma

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

Cite this

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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.",
keywords = "Ataxia, Calcium channel, Lambert-Eaton syndrome, Myasthenic syndrome, Paraneoplastic, Small-cell lung carcinoma",
author = "Nicholas Zalewski and Lennon, {Vanda A} and Pittock, {Sean J} and McKeon, {Andrew B}",
year = "2018",
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doi = "10.1002/mus.26053",
language = "English (US)",
journal = "Muscle and Nerve",
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T1 - Calcium channel autoimmunity

T2 - Cerebellar ataxia and lambert-eaton syndrome coexisting

AU - Zalewski, Nicholas

AU - Lennon, Vanda A

AU - Pittock, Sean J

AU - McKeon, Andrew B

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - Ataxia

KW - Calcium channel

KW - Lambert-Eaton syndrome

KW - Myasthenic syndrome

KW - Paraneoplastic

KW - Small-cell lung carcinoma

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