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Muscle Nerve. 2015 Sep;52(3):386-91. doi: 10.1002/mus.24559. Epub 2015 Jun 30.

Clinical experience of seropositive ganglionic acetylcholine receptor antibody in a tertiary neurology referral center.

Author information

1
Department of Neurology, Desk S90, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio, 44195, USA.
2
Department of Quantitative Health Sciences, Learner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
3
Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Abstract

INTRODUCTION:

Antibody against the acetylcholine receptor of autonomic ganglia (gAChR-Ab) is implicated in the pathogenesis of autoimmune autonomic ganglionopathy (AAG) and several other disorders.

METHODS:

This study was a retrospective evaluation of 95 patients positive for gAChR-Ab.

RESULTS:

Twenty-one (22%) patients had AAG, with a greater median gAChR-Ab level (0.21 nmol/L) and higher percentage (57%) of antibody levels >0.20 nmol/L when compared with the remaining 74 patients without autonomic manifestations (non-AAG group, 0.10 nmol/L and 15%, respectively). Only 2 new cases of malignancy were diagnosed after gAChR-Ab detection. The non-AAG group was associated with high frequencies of neurological and non-neurological autoimmunity, but also included 23 (31%) patients with mostly degenerative disorders.

CONCLUSION:

Detection of gAChR-Ab, especially at a higher level, is helpful for the diagnosis of AAG in patients with corresponding autonomic symptoms. However, its value is limited for predicting cancer risk and for diagnosis and management of patients without autonomic symptoms.

KEYWORDS:

autoimmune autonomic ganglionopathy; autoimmunity; diagnostic testing; ganglionic autonomic acetylcholine receptor; malignancy

PMID:
25557122
DOI:
10.1002/mus.24559
[Indexed for MEDLINE]

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