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Review
. 2019 Jun;32(3):475-483.
doi: 10.1097/WCO.0000000000000684.

Management of neurosarcoidosis: a clinical challenge

Mareye Voortman  1   2   3 , Marjolein Drent  1   3   4 , Robert P Baughman  5
Affiliations
Review

Management of neurosarcoidosis: a clinical challenge

Mareye Voortman et al. Curr Opin Neurol. 2019 Jun.

Abstract

Purpose of review: Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality.

Recent findings: Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease. Recent guidelines have helped to clarify criteria for diagnosing neurosarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment depends on the presentation and distribution, extensiveness, and severity of neurosarcoidosis. As regards evidence-based treatment, only a few randomized controlled trials have been done. Hence, several aspects of (neuro)sarcoidosis management are not fully addressed by the current literature.

Summary: Significant advances have been made in the potential and accuracy of diagnostics for neurosarcoidosis. Treatment should be approached within the context of the patient's anticipated clinical course, avoidance of adverse drug effects, and, if necessary, from the perspective of the comprehensive management of a chronic disease. A multidisciplinary approach to the management of sarcoidosis is strongly recommended.

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Figures

Box 1
Box 1
no caption available
FIGURE 1
FIGURE 1
Approach to diagnosis of neurosarcoidosis.
FIGURE 2
FIGURE 2
A 36-year-old man, presenting with numbness in both arms and cervical pain. MRI showed a cervical myelitis (C3–C5, arrows). Bilateral mediastinal lymph-adenopathy was seen on a chest X-ray and the diagnosis sarcoidosis was confirmed with an endobronchial ultrasound fine-needle aspiration (EBUS-FNA).
FIGURE 3
FIGURE 3
Stepwise treatment for neurosarcoidosis. RCI, repository corticotropin injection. ∗indicates drugs that have been reported specifically for neurosarcoidosis. †indicates therapies which may be used for severe or progressive disease. See text for further details.
See this image and copyright information in PMC

References

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