Neurosarcoidosis: Phenotypes, Approach to Diagnosis and Treatment

Jeanne Gosselin; Chantal Roy-Hewitson; Sean S M Bullis; John C DeWitt; Bruno P Soares; Sidarth Dasari; Alana Nevares

doi:10.1007/s11926-022-01089-z

Neurosarcoidosis: Phenotypes, Approach to Diagnosis and Treatment

Curr Rheumatol Rep. 2022 Dec;24(12):371-382. doi: 10.1007/s11926-022-01089-z. Epub 2022 Oct 12.

Authors

Jeanne Gosselin¹, Chantal Roy-Hewitson², Sean S M Bullis³, John C DeWitt⁴, Bruno P Soares⁵, Sidarth Dasari⁶, Alana Nevares⁷

Affiliations

¹ Division of Rheumatology and Clinical Immunology, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, 111 Colchester Avenue, Burlington, VT, 05401, USA. Jeanne.Gosselin2@uvmhealth.org.
² Department of Neurosciences, Division of Neuroimmunology, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, Burlington, VT, USA.
³ Division of Infectious Disease, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, Burlington, VT, USA.
⁴ Department of Pathology and Laboratory Medicine, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, Burlington, VT, USA.
⁵ Division of Neuroradiology, Department of Radiology, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, Burlington, VT, USA.
⁶ Department of Neurosciences, Division of Neuroimmunology, University of Vermont Medical Center, Burlington, VT, USA.
⁷ Division of Rheumatology and Clinical Immunology, Robert Larner, MD College of Medicine at the University of Vermont and University of Vermont Medical Center, 111 Colchester Avenue, Burlington, VT, 05401, USA.

PMID: 36223002
DOI: 10.1007/s11926-022-01089-z

Abstract

Purpose of review: The aim of this review is to provide an update of clinical presentation, diagnosis, differential diagnoses, and treatment according to recent evidence.

Recent findings: Neurosarcoidosis remains a diagnosis of exclusion, with infectious and malignant etiologies recognized as important mimickers. Corticosteroids remain as first-line therapy. In recent years, however, studies have demonstrated the effectiveness of anti-tumor necrosis factor (anti-TNF) therapy in the treatment of neurosarcoidosis, leading to improved outcomes. Neurosarcoidosis is a granulomatous disease with protean manifestations that may affect any part of the central and peripheral nervous system. It has many mimickers, and potentially devastating complications necessitating long-term follow-up. Early initiation of treatment, particularly with anti-TNF therapy, may lead to better outcomes and fewer relapses. There is an unmet need for randomized controlled trials that provide robust data to guide therapy and the long-term management of neurosarcoidosis patients.

Keywords: CNS; Neurosarcoidosis; Sarcoidosis; Treatment.

Publication types

Review

MeSH terms

Central Nervous System Diseases* / diagnosis
Central Nervous System Diseases* / drug therapy
Humans
Phenotype
Sarcoidosis* / diagnosis
Sarcoidosis* / drug therapy
Tumor Necrosis Factor Inhibitors

Substances

Tumor Necrosis Factor Inhibitors

Supplementary concepts

Neurosarcoidosis