Semin Respir Crit Care Med 2020; 41(05): 641-651
DOI: 10.1055/s-0040-1710576
Review Article

The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment

Mareye Voortman
1  Division of Heart and Lungs, Department of Pulmonology, University Medical Centre Utrecht, The Netherlands
2  ILD Care Foundation Research Team, Ede, The Netherlands
,
Barney J. Stern
3  Department of Neurology, Johns Hopkins University, Baltimore, Maryland
,
Lesley Ann Saketkoo
4  New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, Louisiana
5  Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, University Medical Center, New Orleans, Louisiana
6  Louisiana State University and Tulane University Schools of Medicine, New Orleans, Louisiana
,
Marjolein Drent
2  ILD Care Foundation Research Team, Ede, The Netherlands
7  Department of Pharmacology and Toxicology, FHML, Maastricht University, Maastricht, The Netherlands
8  Department of Pulmonology, ILD Center of Excellence, St. Antonius Hospital, Nieuwegein, The Netherlands
› Author Affiliations

Abstract

Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations. However, non-organ-specific potentially neurologically driven symptoms, such as fatigue, cognitive dysfunction, and small fiber neuropathy, appear frequently.

Heterogeneous clinical presentations and absence of any single conclusive test or biomarker render NS, and sarcoidosis itself, a challenging definitive diagnosis. Clinical suspicion of NS warrants a thorough systemic and neurologic evaluation hopefully resulting in supportive extraneural physical exam and/or tissue findings. Treatment targets the severity of the manifestation, with careful discernment of whether NS reflects active potentially reversible inflammatory granulomatous disease versus inactive postinflammatory damage whereby functional impairment is unlikely to be pharmacologically responsive. Non-organ-specific symptoms are poorly understood, challenging in deciphering reversibility and often identified too late to respond to conventional immunosuppressive/pharmacological treatment. Physical therapy, coping strategies, and stress reduction may benefit patients with all disease activity levels of NS.

This publication provides an approach to screening, diagnosis, disease activity discernment, and pharmacological as well as nonpharmacological treatment interventions to reduce disability and protect health-related quality of life in NS.

Supplementary Material



Publication History

Publication Date:
10 August 2020 (online)

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