Autoimmune autonomic ganglionopathy: Treatment by plasma exchanges and rituximab

Richard Imrich; Steven Vernino; Basil A. Eldadah; Courtney Holmes; David S. Goldstein

doi:10.1007/s10286-009-0012-7

Autoimmune autonomic ganglionopathy: Treatment by plasma exchanges and rituximab

Richard Imrich, Steven Vernino, Basil A. Eldadah, Courtney Holmes, David S. Goldstein

Research output: Contribution to journal › Article › peer-review

39 Scopus citations

Abstract

This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.

Original language	English (US)
Pages (from-to)	259-262
Number of pages	4
Journal	Clinical Autonomic Research
Volume	19
Issue number	4
DOIs	https://doi.org/10.1007/s10286-009-0012-7
State	Published - Aug 2009

Keywords

Autoimmune autonomic ganglionopathy
Plasma exchange
Pure autonomic failure
Rituximab

ASJC Scopus subject areas

Endocrine and Autonomic Systems
Clinical Neurology

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10.1007/s10286-009-0012-7

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title = "Autoimmune autonomic ganglionopathy: Treatment by plasma exchanges and rituximab",

abstract = "This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.",

keywords = "Autoimmune autonomic ganglionopathy, Plasma exchange, Pure autonomic failure, Rituximab",

author = "Richard Imrich and Steven Vernino and Eldadah, {Basil A.} and Courtney Holmes and Goldstein, {David S.}",

note = "Funding Information: Acknowledgments The authors acknowledge Dr. Kaho Wong, MD who administered rituximab to the patient. The authors thank clinical support personnel of the Clinical Neurocardiology Section, including Sandra Pechnik, RN, LaToya Sewell, CRNP, and Tereza Jenkins. The study was supported by the intramural research program of the NINDS.",

year = "2009",

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doi = "10.1007/s10286-009-0012-7",

language = "English (US)",

volume = "19",

pages = "259--262",

journal = "Clinical Autonomic Research",

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T2 - Treatment by plasma exchanges and rituximab

AU - Imrich, Richard

AU - Vernino, Steven

AU - Eldadah, Basil A.

AU - Holmes, Courtney

AU - Goldstein, David S.

N1 - Funding Information: Acknowledgments The authors acknowledge Dr. Kaho Wong, MD who administered rituximab to the patient. The authors thank clinical support personnel of the Clinical Neurocardiology Section, including Sandra Pechnik, RN, LaToya Sewell, CRNP, and Tereza Jenkins. The study was supported by the intramural research program of the NINDS.

PY - 2009/8

Y1 - 2009/8

N2 - This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.

AB - This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.

KW - Autoimmune autonomic ganglionopathy

KW - Plasma exchange

KW - Pure autonomic failure

KW - Rituximab

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Autoimmune autonomic ganglionopathy: Treatment by plasma exchanges and rituximab

Abstract

Keywords

ASJC Scopus subject areas

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