An elderly African American woman presented to our clinic following 9 months of right-sided unilateral headache, otorrhoea and progressive hearing loss. Despite treatment with topical and oral antibiotics, her clinical condition worsened, and imaging showed mastoid coalescence with an associated subgaleal abscess. She underwent right mastoidectomy and was discharged 3 days later on broad-spectrum intravenous antibiotics despite negative operative cultures. Six weeks later, she was hospitalised with diplopia secondary to a right lateral rectus palsy. Imaging showed abscess resolution but progressive bony remodelling and enhancement of the lateral extending into anterior skull base. Chest CT demonstrated upper lobe predominant pulmonary micronodules, and mastoid biopsy on revision surgery was notable for non-caseating granulomas. Further extensive work-up could not identify an alternative cause, and a presumptive diagnosis of neurosarcoidosis was made. The patient was initiated on intravenous steroids, experienced symptomatic improvement and was thereafter transitioned to oral steroid taper on discharge.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7607583PMC
http://dx.doi.org/10.1136/bcr-2020-237676DOI Listing

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Article Synopsis
  • - The case discusses a 64-year-old woman with a complex set of symptoms including difficulty speaking and swallowing, which led to the consideration of various potential diagnoses, including neurosarcoidosis.
  • - Diagnostic procedures revealed cranial nerve palsies; while MRI was inconclusive, CT scans indicated vocal cord paralysis, and lab tests showed elevated CSF biomarker levels, supporting the diagnosis of neurosarcoidosis.
  • - Treatment with steroids and infliximab showed significant improvement in her symptoms and highlights the importance of CSF sIL-2R as a useful biomarker while also promoting the use of steroid-sparing therapies in managing the condition.
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Objective: To evaluate the cerebrospinal fluid (CSF) cytokine/chemokine profile of central nervous system (CNS) neurosarcoidosis (NS), and its utility in differential diagnosis, treatment, and prognostication.

Methods: In this case-control study, we validated 17 cytokines/chemokines (interleukin [IL]-1-beta, IL-2, IL-4, IL-5, IL-6, IL-10, IL-12p70, IL-13, IL-17A, BAFF, IL-8/CXCL8, CXCL9, CXCL10, CXCL13, GM-CSF, interferon-gamma, and tumor necrosis factor [TNF]-alpha) in a multiplexed automated immunoassay system (ELLA; Bio-Techne, Minneapolis, MN, USA), and assessed them in CSF and serum of symptomatic patients with probable or definite CNS NS (01/2011-02/2023) with gadolinium enhancement and/or CSF pleocytosis. Patients with multiple sclerosis, primary CNS lymphoma, aquaporin-4 immunoglobulin G positivity, non-inflammatory disorders, and healthy individuals were used as controls.

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Background: Sarcoidosis, a multisystem inflammatory non-caseating granulomatous disease, can present with neurologic lesions in up to 10% of patients.

Case Description: A 57-year-old male presented with three months of worsening upper extremity radicular pain associated with dysmetria, hyperreflexia, bilateral Hoffman's, and positive Babinski signs. The contrast magnetic resonance imaging (MRI) showed a diffuse T2 signal hyperintensity and T1-enhancing 2.

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Reevaluating the relevance of F-FDG PET findings for diagnosis of neurosarcoidosis: a case series.

Neurol Res Pract

January 2024

Department of Neurology and Experimental Neurology, Charité Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.

Objective: The diagnosis of neurosarcoidosis (NS) remains challenging due to the difficulty to obtain central nervous system (CNS) biopsies. Various diagnostic parameters are considered for the definition of possible, probable and definite NS. Magnetic resonance imaging (MRI) is the imaging gold standard and considered in diagnostic criteria.

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Cardiac Involvement in Neurosarcoidosis: A Single-Center Investigation.

Neurol Neuroimmunol Neuroinflamm

March 2024

From the Department of Neurology (S.N.G., K.-H.W., T.F., M.A.W., J.L., T.S., S.L.C.); Division of Cardiovascular Medicine (J.S., L.K.), Department of Internal Medicine, University of Utah; Department of Neurology (J.S., L.K., T.S., S.L.C.); Geriatric Research Education and Clinical Center (T.S.), George E. Whalen Department of Veterans Affairs Medical Center; and Department of Pathology (T.S.), University of Utah, Salt Lake City.

Background And Objectives: Sarcoidosis is a multisystem inflammatory granulomatous disease. Among systemic sarcoidosis manifestations, cardiac or nervous system involvement can result in significant morbidity and mortality. We describe the overlapping incidence of cardiac sarcoidosis (CS) within a neurosarcoidosis (NS) cohort and determine the frequency of other nonsarcoid cardiac diseases in these patients.

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