Neurosarcoidosis according to Zajicek and Scolding criteria: 15 probable and definite cases, their treatment and outcomes

Cação, Gonçalo; Branco, Ana; Meireles, Mariana; Alves, José Eduardo; Mateus, Andrea; Silva, Ana Martins; Santos, Ernestina

doi:10.1016/j.jns.2017.05.055

Cited by 19 publications

(11 citation statements)

References 22 publications

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“…Evidence of systemic sarcoidosis in this patient included hilar lymphadenopathy and a history of uveitis and keratitis, although these findings do not conform to all the proposed criteria of Zajicek et al for the diagnosis of neurosarcoidosis [4, 10]. Central nervous system studies suggestive of neurosarcoidosis included leptomeningeal enhancement, elevated proteins in the CSF, and CSF lymphocytic pleocytosis [2, 4, 11]. No oligoclonal bands were found.…”

Section: Discussionmentioning

confidence: 54%

“…Extensive studies during multiple hospitalizations did not find alternative infectious and neoplastic pathologies to fully account for the patient's symptoms and findings, thereby lending credence to neurosarcoidosis as a diagnosis of exclusion. Additionally, the patient had clinical improvement on methotrexate and infliximab and previously corticosteroids, which are typical treatments for neurosarcoidosis [2, 11, 14].…”

Section: Discussionmentioning

confidence: 99%

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Disseminated Histoplasmosis with Miliary Histoplasmosis, Neurohistoplasmosis, and Histoplasma capsulatum Bacteremia in Probable Neurosarcoidosis

Bui

2018

Case Reports in Medicine

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Introduction Neurosarcoidosis, either isolated or as part of systemic sarcoidosis, is an uncommon entity and has diagnostic uncertainty. Treatment for neurosarcoidosis can increase the risk of infections, including fungal infections such as disseminated histoplasmosis. Neurosarcoidosis may further predispose patients to infections of the central nervous system. Case Presentation A 54-year-old male with a history of probable neurosarcoidosis on methotrexate and infliximab presented with encephalopathy, hypoxia, and reported fevers. The patient was found to have disseminated histoplasmosis involving the lungs (miliary histoplasmosis), central nervous system (neurohistoplasmosis), and bloodstream. The Histoplasma capsulatum infection was treated with amphotericin and then voriconazole. Discussion Patients with neurosarcoidosis are suspected to have blood-brain barrier dysfunction. Lumbar puncture should be considered as part of initial investigative studies for infection. Empiric antimicrobial therapy for a patient with neurosarcoidosis on immunosuppressive agents may need to include antifungal agents.

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Section: Discussionmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 99%

Disseminated Histoplasmosis with Miliary Histoplasmosis, Neurohistoplasmosis, and Histoplasma capsulatum Bacteremia in Probable Neurosarcoidosis

Bui

2018

Case Reports in Medicine

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“…The criteria for probable neurosarcoidosis: clinical symptoms and diagnostical evaluation suggest to neurosarcoidosis. The alternative diagnosis is excluded and there is a histological conformation of systemic sarcoidosis [10,11].…”

Section: How To Diagnose Neurosarcoidosis?mentioning

confidence: 99%

Neurosarcoidosis - an ever-present clinical challenge

et al. 2021

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Sarcoidosis afflicts the central nervous system more frequently than previously believed. Neurological symptoms are present in roughly a half of patients, and depend of the location in central nervous system. The probability of spontaneous regression is significantly less when compared to other forms of sarcoidosis, which means that the proper diagnosis and treatment is paramount. Even when properly treated, functional defects are not uncommon. Majority of these patients require immunomodulating drugs and continuous follow-up. New immunomodulating drugs, especially biological agents, have shown to be significantly more effective, with fewer side effects, and are important when corticosteroids could not be applied. Less invasive methods, such as cerebrospinal analysis, help greatly in the diagnostics procedure, and require further research and improvement

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“… 2 , 3 Clinical features of NS include, among other things, cranial neuropathy, seizure, aseptic meningitis, hydrocephalus and myelitis. 2 – 4 …”

Section: Introductionmentioning

confidence: 99%

“…Magnetic resonance imaging (MRI) often shows leptomeningeal involvement of the basilar meninges but virtually any portion of the CNS may be affected. 2 , 4 Currently, no reliable serologic marker exists. Laboratory testing includes serum angiotensin converting enzyme and soluble interleukin-2 receptor (sIL-2R) but both may also be negative in patients with biopsy proof of NS.…”

Section: Introductionmentioning

confidence: 99%

Successful therapy with rituximab in three patients with probable neurosarcoidosis

Zella

Kneiphof

Haghikia

et al. 2018

Ther Adv Neurol Disord

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Background:Neurosarcoidosis occurs in about 5–15% of patients with sarcoidosis. Therapy with corticosteroids is generally accepted as the first-line medication, followed by various immunomodulating and cytotoxic agents or combined therapy. However, some patients show an unsatisfactory outcome or have adverse events and require novel treatment strategies.Methods:We describe three patients with systemic sarcoidosis and central nervous system involvement who received CD20-targeted B-cell depletion with rituximab.Results:Treatment with rituximab was well tolerated and followed by marked remission in patients nonresponsive to other immunosuppressive agents.Conclusion:Rituximab may be used for patients with neurosarcoidosis who are nonresponsive to established treatment regimes.

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Neurosarcoidosis according to Zajicek and Scolding criteria: 15 probable and definite cases, their treatment and outcomes

Cited by 19 publications

References 22 publications

Disseminated Histoplasmosis with Miliary Histoplasmosis, Neurohistoplasmosis, and Histoplasma capsulatum Bacteremia in Probable Neurosarcoidosis

Disseminated Histoplasmosis with Miliary Histoplasmosis, Neurohistoplasmosis, and Histoplasma capsulatum Bacteremia in Probable Neurosarcoidosis

Neurosarcoidosis - an ever-present clinical challenge

Successful therapy with rituximab in three patients with probable neurosarcoidosis

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