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July 2020; 7 (4) ArticleOpen Access

Neurosarcoidosis

Longitudinal experience in a single-center, academic healthcare system

Jennifer Lord, M. Mateo Paz Soldan, Jonathan Galli, Karen L. Salzman, Jacob Kresser, Rae Bacharach, L. Dana DeWitt, Julia Klein, John Rose, John Greenlee, Stacey L. Clardy
First published May 13, 2020, DOI: https://doi.org/10.1212/NXI.0000000000000743
Jennifer Lord
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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M. Mateo Paz Soldan
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Jonathan Galli
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Karen L. Salzman
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Jacob Kresser
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Rae Bacharach
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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L. Dana DeWitt
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Julia Klein
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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John Rose
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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John Greenlee
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Stacey L. Clardy
From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
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Citation
Neurosarcoidosis
Longitudinal experience in a single-center, academic healthcare system
Jennifer Lord, M. Mateo Paz Soldan, Jonathan Galli, Karen L. Salzman, Jacob Kresser, Rae Bacharach, L. Dana DeWitt, Julia Klein, John Rose, John Greenlee, Stacey L. Clardy
Neurol Neuroimmunol Neuroinflamm Jul 2020, 7 (4) e743; DOI: 10.1212/NXI.0000000000000743

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Abstract

Objective To characterize patients with neurosarcoidosis within the University of Utah healthcare system, including demographics, clinical characteristics, treatment, and long-term outcomes.

Methods We describe the clinical features and outcomes of patients with neurosarcoidosis within the University of Utah healthcare system (a large referral center for 10% of the continental United States by land mass). Patients were selected who met the following criteria: (1) at least one International Classification of Diseases Clinical Modification, 9th revision code 135 or International Classification of Diseases Clinical Modification, 10th revision code D86* (sarcoidosis) and (2) at least one outpatient visit with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record.

Results We identified 56 patients meeting the study criteria. Thirty-five patients (63%) were women, and most patients (84%) were white. Twelve patients (22%) met the criteria for definite neurosarcoidosis, 36 patients (64%) were diagnosed with probable neurosarcoidosis, and 8 patients (14%) were diagnosed with possible neurosarcoidosis. A total of 8 medications were used for the treatment of neurosarcoidosis. Prednisone was the first-line treatment in 51 patients (91%). Infliximab was the most effective therapy, with 87% of patients remaining stable or improving on infliximab. Treatment response for methotrexate and azathioprine was mixed, and mycophenolate mofetil and rituximab were the least effective treatments in this cohort.

Conclusions This is a comprehensive characterization of neurosarcoidosis within a single healthcare system at the University of Utah that reports long-term response to treatment and outcomes of patients with neurosarcoidosis. Our results suggest the use of infliximab as a first-line therapy for neurosarcoidosis.

GLOSSARY

ACE=
angiotensin-converting enzyme;
CVID=
common variable immune deficiency;
PNS=
peripheral nervous system;
TNFα=
tumor necrosis factor-alpha

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by the authors.

  • Received February 18, 2020.
  • Accepted in final form April 13, 2020.
  • Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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