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Autoimmunity-associated autonomic failure with sympathetic denervation

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Abstract

We report a case of autoimmunity-associated autonomic failure in a young adult woman who developed arthritis followed 3 years later by pandysautonomia. There was early recovery of parasympathetic functions but persistent neurogenic orthostatic hypotension from post-ganglionic sympathetic denervation. Clinical laboratory testing indicated variable amounts of sympathetic neuronal re-sprouting in the heart, kidneys, eyes, and body as a whole upon follow-up evaluation after 1.5 years.

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Abbreviations

AAG:

Autoimmune autonomic ganglionopathy

ANA:

Anti-nuclear antibody

CSF:

Cerebrospinal fluid

DHPG:

3,4-Dihydroxyphenylglycol

DOPA:

3,4-Dihydroxyphenylalanine

DOPAC:

3,4-Dihydroxyphenylacetic acid

EPI:

Epinephrine

18F-DA:

18F-Dopamine

GBS:

Guillain–Barré syndrome

IV:

Intravenous

IVIG:

Intravenous immunoglobulin

NE:

Norepinephrine

OH:

Orthostatic hypotension

PAF:

Pure autonomic failure

PET:

Positron emission tomography

QSART:

Quantitative sudomotor axon reflex test

References

  1. Appenzeller O, Kornfeld M (1973) Acute pandysautonomia. Clin and morphologic study. Arch Neurol 29:334–339

    Article  CAS  PubMed  Google Scholar 

  2. Goldstein DS, Holmes C, Dendi R, Li ST, Brentzel S, Vernino S (2002) Pandysautonomia associated with impaired ganglionic neurotransmission and circulating antibody to the neuronal nicotinic receptor. Clin Auton Res 12:281–285

    Article  PubMed  Google Scholar 

  3. Goldstein DS, Holmes C, Frank SM, Naqibuddin M, Dendi R, Snader S, Calkins H (2003) Sympathoadrenal imbalance before neurocardiogenic syncope. Am J Cardiol 91:53–58

    Article  PubMed  Google Scholar 

  4. Tohgi H, Sano M, Sasaki K, Suzuki H, Sato T, Iwasaki T, Satodate R (1989) Acute autonomic and sensory neuropathy: report of an autopsy case. Acta Neuropathol 77:659–663

    Article  CAS  PubMed  Google Scholar 

  5. Young RR, Asbury AK, Adams RD, Corbett JL (1969) Pure pan-dysautonomia with recovery. Trans Am Neurol Assoc 94:355–357

    CAS  PubMed  Google Scholar 

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Acknowledgements

Funding was provided by the National Institute of Neurological Disorders and Stroke (NINDS), project number 1ZIANS003034.

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Authors and Affiliations

  1. Clinical Neurocardiology Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 9000 Rockville Pike MSC-1620, Building 10 Room 5N220, Bethesda, MD, 20892-1620, USA

    David S. Goldstein, Courtney Holmes, Patti Sullivan, Risa Isonaka, Elizabeth Zhong, Bobby Pourier & Irwin J. Kopin

  2. IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy

    Vincenzo Donadio

  3. University of Texas Southwestern Medical Center, Dallas, TX, USA

    Steven Vernino

  4. Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel

    Yehonatan Sharabi

  5. Chaim Sheba Medical Center, Tel-Hashomer, Israel

    Yehonatan Sharabi

Authors
  1. David S. Goldstein
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  2. Courtney Holmes
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  3. Patti Sullivan
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  4. Vincenzo Donadio
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  5. Risa Isonaka
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  6. Elizabeth Zhong
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  7. Bobby Pourier
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  8. Steven Vernino
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  9. Irwin J. Kopin
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  10. Yehonatan Sharabi
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Corresponding author

Correspondence to David S. Goldstein.

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On behalf of all authors, the corresponding author states that there is no conflict of interest.

Financial support

Division of Intramural Research, NINDS, NIH.

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Cite this article

Goldstein, D.S., Holmes, C., Sullivan, P. et al. Autoimmunity-associated autonomic failure with sympathetic denervation. Clin Auton Res 27, 57–62 (2017). https://doi.org/10.1007/s10286-016-0388-0

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  • DOI: https://doi.org/10.1007/s10286-016-0388-0

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