Management of neurosarcoidosis: a clinical challenge

Voortman, Mareye; Drent, Marjolein; Baughman, Robert P.

doi:10.1097/wco.0000000000000684

Cited by 68 publications

(73 citation statements)

References 71 publications

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“…Treatment recommendations include a stepwise approach based on the severity of the disease. This typically begins with oral glucocorticoids and progresses to high-dose oral or intravenous glucocorticoids for severe or progressive disease [14]. The next line of treatment involves steroid-sparing agents, such as methotrexate or mycophenolate.…”

Section: Discussionmentioning

confidence: 99%

Isolated Neurosarcoidosis Mimicking Demyelination and Metastases

Domonkos

Shuster

2019

SN Compr. Clin. Med.

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Involvement of the central nervous system (CNS) is commonly seen in patients with systemic sarcoidosis; however, an isolated case of neurosarcoidosis is a substantially infrequent scenario. Neurosarcoidosis may present with numerous nonspecific clinical manifestations and radiological features, which makes it difficult to diagnose. Correct interpretation of magnetic resonance imaging (MRI) findings incorporating sarcoidosis as a possible diagnosis is important for timely management of the disease. We report the unusual case of a 47-year-old female with brain lesions and a sudden appearance of spinal lesions mimicking CNS demyelination and metastases. Biopsy provided a definitive diagnosis of neurosarcoidosis.

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Section: Discussionmentioning

confidence: 99%

Isolated Neurosarcoidosis Mimicking Demyelination and Metastases

Domonkos

Shuster

2019

SN Compr. Clin. Med.

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“…In all patients with suspected NS a thorough neurologic evaluation is essential. 57 Gadoliniumenhanced magnetic resonance imaging (MRI) is the imaging modality of choice for investigating enhancing brain or spinal cord parenchymal lesions, nonenhancing WMLs, dural thickening or mass, pia-arachnoid enhancement, and cranial nerve enhancement (►Figs. 1 and 2).…”

Section: Diagnostic Approach Neurosarcoidosis Manifestationsmentioning

confidence: 99%

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

Voortman

Stern

Saketkoo

et al. 2020

Semin Respir Crit Care Med

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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.

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“…Im klinischen Alltag findet ein Stufenschema Verwendung, in dem als Erstlinientherapie Kortikosteroide eingesetzt werden, wobei sich die von unterschiedlicher Seite empfohlenen Dosierungen zum Teil unterscheiden [38].…”

Section: Therapie Der Neurosarkoidoseunclassified

“…mit anschließender oraler Erhaltungstherapie erfolgen [38], was von uns meist präferiert wird. Bei Unverträglichkeiten gegen Infliximab kann ggf.…”

Section: Therapie Der Neurosarkoidoseunclassified