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

Mareye Voortman*, Barney J. Stern, Lesley Ann Saketkoo, Marjolein Drent

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

3 Citations (Web of Science)

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 thoroughsystemicand 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.

Original languageEnglish
Pages (from-to)641-651
Number of pages11
JournalSeminars in Respiratory and Critical Care Medicine
Volume41
Issue number05
DOIs
Publication statusPublished - Oct 2020

Keywords

  • neurosarcoidosis
  • sarcoidosis
  • paralysis
  • peripheral neuropathy
  • small fiber neuropathy
  • fatigue
  • cognition
  • SMALL-FIBER NEUROPATHY
  • CORNEAL CONFOCAL MICROSCOPY
  • CENTRAL-NERVOUS-SYSTEM
  • EVERYDAY COGNITIVE FAILURE
  • LONG-TERM OUTCOMES
  • QUALITY-OF-LIFE
  • BRONCHOALVEOLAR LAVAGE
  • CNS SARCOIDOSIS
  • SKIN BIOPSY
  • FATIGUE

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