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Susan Lightman - One of the best experts on this subject based on the ideXlab platform.

  • ocular features in Neurosarcoidosis
    Ocular Immunology and Inflammation, 2009
    Co-Authors: Victor Menezo, Aires Lobo, Tun Kuan Yeo, Roland M Du Bois, Susan Lightman
    Abstract:

    Background/aims: To determine the type of ocular involvement in patients with Neurosarcoidosis, and evaluate whether the type of eye involvement may help in the diagnosis of Neurosarcoidosis. Methods: Retrospective, case history study. We reviewed the medical records of 46 patients who attended the sarcoidosis clinics at the Royal Brompton and Moorfields Eye Hospital over a 4-year period with a diagnosis of definite and probable Neurosarcoidosis supported by laboratory investigations and exclusion of other causes for the neurological symptoms. Results: Cranial nerve involvement was the most common neurological manifestation in this series. Among the 27 patients with cranial neuropathy, lower motor neurone facial palsy was the most frequently seen in 19 patients (70.4%). Diplopia was seen in four patients (14.9%). In three patients, this was because of common oculomotor nerve paresis. Uveitis was the most common intraocular manifestation in patients with Neurosarcoidosis. The majority of these patients (9,...

  • Ocular Features in Neurosarcoidosis
    Ocular immunology and inflammation, 2009
    Co-Authors: Victor Menezo, Aires Lobo, Tun Kuan Yeo, Roland M Du Bois, Susan Lightman
    Abstract:

    To determine the type of ocular involvement in patients with Neurosarcoidosis, and evaluate whether the type of eye involvement may help in the diagnosis of Neurosarcoidosis. Retrospective, case history study. We reviewed the medical records of 46 patients who attended the sarcoidosis clinics at the Royal Brompton and Moorfields Eye Hospital over a 4-year period with a diagnosis of definite and probable Neurosarcoidosis supported by laboratory investigations and exclusion of other causes for the neurological symptoms. Cranial nerve involvement was the most common neurological manifestation in this series. Among the 27 patients with cranial neuropathy, lower motor neurone facial palsy was the most frequently seen in 19 patients (70.4%). Diplopia was seen in four patients (14.9%). In three patients, this was because of common oculomotor nerve paresis. Uveitis was the most common intraocular manifestation in patients with Neurosarcoidosis. The majority of these patients (9, 64.3%) suffered from anterior uveitis, but in 35.7% of them the inflammatory process involved the posterior segment. We found a higher incidence of ocular manifestations, including intraocular inflammation in Neurosarcoidosis compared to that in systemic sarcoidosis elsewhere. The most common ocular complication seen in our series was anterior uveitis; however there were no associated clinical features of the uveitis in these series that could contribute to the differential diagnosis between Neurosarcoidosis and other autoimmune disorders with neuro-ophthalmic features such as multiple sclerosis. Patients with neurological symptoms and associated intraocular inflammation should have a routine work-up for sarcoidosis. Investigations should include MRI scan of the brain and orbits and lumbar puncture in selected cases. Tissue biopsy should be attempted when clinically accessible lesions are available i.e., conjunctiva or lacrimal gland.

Matthijs C. Brouwer - One of the best experts on this subject based on the ideXlab platform.

  • Clinical features, treatment and outcome in Neurosarcoidosis: systematic review and meta-analysis
    BMC Neurology, 2016
    Co-Authors: Daan Fritz, Diederik Van De Beek, Matthijs C. Brouwer
    Abstract:

    Background Neurosarcoidosis is a rare variant of sarcoidosis and is only described in small cohort studies. We define clinical features, treatment and outcome of patients with Neurosarcoidosis over the last 35 years. Methods We performed a systematic review and meta-analysis of studies on Neurosarcoidosis published between 1980 and 2016. Studies were included if they reported at least 5 cases. Studies describing one specific neurological presentation were excluded. Results We identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of Neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively. First line therapy with corticosteroids was initiated in 434 of 539 patients (81%). Second and third line therapy was started in 27 and 9%. Outcome consisted of complete remission in 27%, incomplete remission in 32%, stable disease in 24%, deterioration in 6% and death in 5%. Conclusion Neurosarcoidosis has a heterogeneous clinical presentation and the diagnosis can be difficult because of low sensitivity of ancillary investigations. New treatments have emerged, but nevertheless one third of patients do not respond to treatment. Prospective cohort studies and RCTs on treatment are urgently needed.

  • clinical features treatment and outcome in Neurosarcoidosis systematic review and meta analysis
    BMC Neurology, 2016
    Co-Authors: Daan Fritz, Diederik Van De Beek, Matthijs C. Brouwer
    Abstract:

    Neurosarcoidosis is a rare variant of sarcoidosis and is only described in small cohort studies. We define clinical features, treatment and outcome of patients with Neurosarcoidosis over the last 35 years. We performed a systematic review and meta-analysis of studies on Neurosarcoidosis published between 1980 and 2016. Studies were included if they reported at least 5 cases. Studies describing one specific neurological presentation were excluded. We identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of Neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively. First line therapy with corticosteroids was initiated in 434 of 539 patients (81%). Second and third line therapy was started in 27 and 9%. Outcome consisted of complete remission in 27%, incomplete remission in 32%, stable disease in 24%, deterioration in 6% and death in 5%. Neurosarcoidosis has a heterogeneous clinical presentation and the diagnosis can be difficult because of low sensitivity of ancillary investigations. New treatments have emerged, but nevertheless one third of patients do not respond to treatment. Prospective cohort studies and RCTs on treatment are urgently needed.

  • Neurosarcoidosis in a Tertiary Referral Center: A Cross-Sectional Cohort Study
    Medicine, 2016
    Co-Authors: Sonja E. Leonhard, Daan Fritz, Filip Eftimov, Anneke J. Van Der Kooi, Diederik Van De Beek, Matthijs C. Brouwer
    Abstract:

    The aim of this study was to evaluate clinical characteristics, diagnostic strategy, and treatment in patients with Neurosarcoidosis in a tertiary referral centre.In a cross-sectional study, we included all patients with Neurosarcoidosis treated at our tertiary referral center between September 2014 and April 2015.We identified 52 patients, among them 1 patient was categorized as having definite Neurosarcoidosis, 37 probable Neurosarcoidosis, and 14 possible Neurosarcoidosis. Neurologic symptoms were the first manifestation of sarcoidosis in 37 patients (71%). Chronic aseptic meningitis was the most common presentation (19/52 patients [37%]), followed by cranial neuropathy (16/52 patients [31%]). Serum angiotensin-converting enzyme and lysozyme levels were elevated in 18 of 41 (44%) and 12 of 26 cases (46%). Pulmonary or lymph node sarcoidosis was identified by chest X-ray in 21 of 39 cases (54%) and by computed tomography of the chest in 25 of 31 cases (81%); Fluorodeoxyglucose-Positron emission tomography showed signs of sarcoidosis in 15 of 19 cases (79%). Thirty-one of the 46 cases receiving treatment (67%) improved, 13 cases (28%) stabilized, and 2 cases (4%) deteriorated. First-line treatment with corticosteroids resulted in satisfactory reduction of symptoms in 21 of 43 patients (49%). Seventeen patients (33%) needed second-line cytostatic treatment, and 10 patients (19%) were treated with tumor necrosis factor-α inhibitors.The majority of patients with Neurosarcoidosis present with chronic meningitis without a history of systemic sarcoidosis. The diagnosis can be difficult to make because of the poor sensitivity of most diagnostic tests. Half of patients had a satisfactory reduction of symptoms on first-line therapy.

Evan Winograd - One of the best experts on this subject based on the ideXlab platform.

  • NIMG-61. UNUSUAL PRESENTATION OF Neurosarcoidosis: A CASE SERIES
    Neuro-Oncology, 2020
    Co-Authors: Bette K. Kleinschmidt-demasters, David Ormond, Evan Winograd
    Abstract:

    Abstract INTRODUCTION Neurosarcoidosis is a rare diagnosis, and even with known systemic disease or history, often remains a diagnosis of exclusion. Typical imaging findings of Neurosarcoidosis include white matter lesions coupled with meningeal enhancement, or “sugarcoating” near the skull base. Occasionally, imaging can be quite unusual and mimic other entities. Other diagnoses to rule out include neoplastic, autoimmune, or infectious pachymeningitis, neoplastic lesions, neurosyphilis or tuberculosis. METHODS Our neuropathology database was queried for Neurosarcoidosis from January 2008 until December 2019. These cases were then reviewed for cases with unusual presentations for further review and discussion. RESULTS Here we present 16 cases of Neurosarcoidosis with histories and/or imaging that did not conform to the typical appearance of Neurosarcoidosis. Along with a rare radiographic presentation, these cases also lacked CSF, laboratory, or systemic findings to suggest a diagnosis of Neurosarcoidosis. 15 of these cases presented intracranially while 1 case presented within the spinal cord. CONCLUSIONS Neurosarcoidosis presentations can vary greatly. A better understanding of some unique patient presentations can help improve noninvasive diagnosis, although biopsy often remains necessary for confirmation.

Zahir Amoura - One of the best experts on this subject based on the ideXlab platform.

  • The eclipse sign as a radiological presentation of Neurosarcoidosis.
    The International journal of neuroscience, 2019
    Co-Authors: Marc Pineton De Chambrun, J. Haroche, Zahir Amoura, Damien Galanaud, Fleur Cohen Aubart
    Abstract:

    Neurosarcoidosis is a rare inflammatory neurological condition. We describe a challenging radiological presentation of Neurosarcoidosis. The eclipse sign refers to hypo T1-weighted parenchymal or leptomeningeal images surrounded by circular gadolinium enhancement. The eclipse sign was identified in 3 out of 46 patients with histologically-proven Neurosarcoidosis. The eclipse sign may correspond to necrotizing parenchymal or leptomeningeal granuloma. This sign expands the spectrum of radiological presentations of Neurosarcoidosis.

  • the cerebrospinal fluid cd4 cd8 ratio and interleukin 6 and 10 levels in Neurosarcoidosis a multicenter pragmatic comparative study
    European Journal of Neurology, 2019
    Co-Authors: T. Chazal, M. Costopoulos, E. Maillart, C. Fleury, D. Psimaras, P. Legendre, J. Haroche, Catherine Lubetzki, Pineton M De Chambrun, Zahir Amoura
    Abstract:

    Background and purpose Neurosarcoidosis is a rare inflammatory disorder of unknown cause. The aim of this study was to evaluate the value of T/B lymphocyte population counts and the concentrations of the cytokines interleukin (IL) 6 and IL-10 in the cerebrospinal fluid (CSF) of Neurosarcoidosis patients. Methods A retrospective study CSF biomarkers was conducted in patients with Neurosarcoidosis who underwent CSF analysis between 2012 and 2017 as well as various control populations. Results Forty-three patients with Neurosarcoidosis, 14 with multiple sclerosis (MS) and 48 with other inflammatory disorders were analyzed. The CSF IL-6 levels were higher in sarcoidosis patients than in MS patients (median 8 vs. 3 pg/ml, P = 0.006). The CSF CD4/CD8 ratio was higher in sarcoidosis patients than in MS patients and in patients with other inflammatory disorders (median 3.18 vs. 2.36 and 2.10, respectively, P = 0.008). The CSF IL-6 level was higher in patients with active Neurosarcoidosis than in non-active Neurosarcoidosis patients (median 13 vs. 3 pg/ml, P = 0.0005). In patients with Neurosarcoidosis, a CSF IL-6 concentration >50 pg/ml was associated with a higher risk of relapse or progression-free survival (hazard ratio 3.60; 95% confidence interval 1.78-23.14). A refractory Neurosarcoidosis patient was treated with an anti-IL-6 monoclonal antibody that produced a complete neurological response. Conclusions The CSF CD4/CD8 ratio and IL-6 concentration are increased in Neurosarcoidosis compared to MS and other inflammatory disorders. A CSF IL-6 concentration >50 pg/ml is associated with relapse or progression of Neurosarcoidosis. IL-10 levels may be elevated in Neurosarcoidosis.

  • infliximab biosimilar for treating Neurosarcoidosis tolerance and efficacy in a retrospective study including switch from the originator and initiation of treatment
    Journal of Neurology, 2019
    Co-Authors: Quentin Riller, J. Haroche, Miguel Hie, Camille Cotteret, Helga Junot, Neila Benameur, Makoto Miyara, Patrick Tilleul, A Mathian, Zahir Amoura
    Abstract:

    Infliximab is increasingly used to treat Neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating Neurosarcoidosis. We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in Neurosarcoidosis patients. We compared the survival time without relapse while receiving the biosimilar or previous originator-infliximab treatment. Twenty patients with histologically documented Neurosarcoidosis were treated with an infliximab biosimilar (initiation of treatment in 12 and switch from the originator drug in 8) between February 2016 and August 2018. All patients presenting with neurological involvement of one or more areas, including meningeal (n = 15), cerebral (n = 10), spinal cord (n = 9), and/or cranial nerves (n = 5); epilepsy (n = 3); and/or intracranial hypertension (n = 3) were enrolled. Eighteen patients received glucocorticoids during infliximab treatment, and 16 had methotrexate or azathioprine concomitant treatment. The median duration of follow-up was 25 months (19–28). Six patients relapsed during biosimilar treatment. Relapse rates and time-to-relapse did not differ between the infliximab originator previously received and biosimilar treatment groups (p = 0.40 and 0.51, respectively). Nine patients experienced 11 adverse events with the infliximab biosimilar, including infections (n = 5), urticaria (n = 4), headache (n = 1), and diarrhea (n = 1). All side effects were grade 2 or less using the WHO classification. In this retrospective study, the infliximab biosimilar was efficacious and safe for treating Neurosarcoidosis.

  • The cerebrospinal fluid CD4/CD8 ratio and interleukin‐6 and ‐10 levels in Neurosarcoidosis: a multicenter, pragmatic, comparative study
    European Journal of Neurology, 2019
    Co-Authors: T. Chazal, M. Costopoulos, E. Maillart, C. Fleury, D. Psimaras, P. Legendre, M. Pineton De Chambrun, J. Haroche, Catherine Lubetzki, Zahir Amoura
    Abstract:

    BACKGROUND AND PURPOSE: Neurosarcoidosis is a rare inflammatory disorder of unknown cause. The aim of this study was to evaluate the value of T/B lymphocyte population counts and the concentrations of the cytokines interleukin (IL) 6 and IL-10 in the cerebrospinal fluid (CSF) of Neurosarcoidosis patients. METHODS: A retrospective study CSF biomarkers was conducted in patients with Neurosarcoidosis who underwent CSF analysis between 2012 and 2017 as well as various control populations. RESULTS: Forty-three patients with Neurosarcoidosis, 14 with multiple sclerosis (MS) and 48 with other inflammatory disorders were analyzed. The CSF IL-6 levels were higher in sarcoidosis patients than in MS patients (median 8 vs. 3 pg/ml, P = 0.006). The CSF CD4/CD8 ratio was higher in sarcoidosis patients than in MS patients and in patients with other inflammatory disorders (median 3.18 vs. 2.36 and 2.10, respectively, P = 0.008). The CSF IL-6 level was higher in patients with active Neurosarcoidosis than in non-active Neurosarcoidosis patients (median 13 vs. 3 pg/ml, P = 0.0005). In patients with Neurosarcoidosis, a CSF IL-6 concentration >50 pg/ml was associated with a higher risk of relapse or progression-free survival (hazard ratio 3.60; 95% confidence interval 1.78-23.14). A refractory Neurosarcoidosis patient was treated with an anti-IL-6 monoclonal antibody that produced a complete neurological response. CONCLUSIONS: The CSF CD4/CD8 ratio and IL-6 concentration are increased in Neurosarcoidosis compared to MS and other inflammatory disorders. A CSF IL-6 concentration >50 pg/ml is associated with relapse or progression of Neurosarcoidosis. IL-10 levels may be elevated in Neurosarcoidosis.

J. Haroche - One of the best experts on this subject based on the ideXlab platform.

  • The eclipse sign as a radiological presentation of Neurosarcoidosis.
    The International journal of neuroscience, 2019
    Co-Authors: Marc Pineton De Chambrun, J. Haroche, Zahir Amoura, Damien Galanaud, Fleur Cohen Aubart
    Abstract:

    Neurosarcoidosis is a rare inflammatory neurological condition. We describe a challenging radiological presentation of Neurosarcoidosis. The eclipse sign refers to hypo T1-weighted parenchymal or leptomeningeal images surrounded by circular gadolinium enhancement. The eclipse sign was identified in 3 out of 46 patients with histologically-proven Neurosarcoidosis. The eclipse sign may correspond to necrotizing parenchymal or leptomeningeal granuloma. This sign expands the spectrum of radiological presentations of Neurosarcoidosis.

  • the cerebrospinal fluid cd4 cd8 ratio and interleukin 6 and 10 levels in Neurosarcoidosis a multicenter pragmatic comparative study
    European Journal of Neurology, 2019
    Co-Authors: T. Chazal, M. Costopoulos, E. Maillart, C. Fleury, D. Psimaras, P. Legendre, J. Haroche, Catherine Lubetzki, Pineton M De Chambrun, Zahir Amoura
    Abstract:

    Background and purpose Neurosarcoidosis is a rare inflammatory disorder of unknown cause. The aim of this study was to evaluate the value of T/B lymphocyte population counts and the concentrations of the cytokines interleukin (IL) 6 and IL-10 in the cerebrospinal fluid (CSF) of Neurosarcoidosis patients. Methods A retrospective study CSF biomarkers was conducted in patients with Neurosarcoidosis who underwent CSF analysis between 2012 and 2017 as well as various control populations. Results Forty-three patients with Neurosarcoidosis, 14 with multiple sclerosis (MS) and 48 with other inflammatory disorders were analyzed. The CSF IL-6 levels were higher in sarcoidosis patients than in MS patients (median 8 vs. 3 pg/ml, P = 0.006). The CSF CD4/CD8 ratio was higher in sarcoidosis patients than in MS patients and in patients with other inflammatory disorders (median 3.18 vs. 2.36 and 2.10, respectively, P = 0.008). The CSF IL-6 level was higher in patients with active Neurosarcoidosis than in non-active Neurosarcoidosis patients (median 13 vs. 3 pg/ml, P = 0.0005). In patients with Neurosarcoidosis, a CSF IL-6 concentration >50 pg/ml was associated with a higher risk of relapse or progression-free survival (hazard ratio 3.60; 95% confidence interval 1.78-23.14). A refractory Neurosarcoidosis patient was treated with an anti-IL-6 monoclonal antibody that produced a complete neurological response. Conclusions The CSF CD4/CD8 ratio and IL-6 concentration are increased in Neurosarcoidosis compared to MS and other inflammatory disorders. A CSF IL-6 concentration >50 pg/ml is associated with relapse or progression of Neurosarcoidosis. IL-10 levels may be elevated in Neurosarcoidosis.

  • Infliximab biosimilar for treating Neurosarcoidosis: tolerance and efficacy in a retrospective study including switch from the originator and initiation of treatment
    Journal of Neurology, 2019
    Co-Authors: Fleur Cohen Aubart, J. Haroche, A Mathian, Miguel Hie, Quentin Riller, Camille Cotteret, Helga Junot, Neila Benameur, Makoto Miyara, Patrick Tilleul
    Abstract:

    OBJECTIVES: Infliximab is increasingly used to treat Neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating Neurosarcoidosis. METHODS: We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in Neurosarcoidosis patients. We compared the survival time without relapse while receiving the biosimilar or previous originator-infliximab treatment. RESULTS: Twenty patients with histologically documented Neurosarcoidosis were treated with an infliximab biosimilar (initiation of treatment in 12 and switch from the originator drug in 8) between February 2016 and August 2018. All patients presenting with neurological involvement of one or more areas, including meningeal (n = 15), cerebral (n = 10), spinal cord (n = 9), and/or cranial nerves (n = 5); epilepsy (n = 3); and/or intracranial hypertension (n = 3) were enrolled. Eighteen patients received glucocorticoids during infliximab treatment, and 16 had methotrexate or azathioprine concomitant treatment. The median duration of follow-up was 25 months (19-28). Six patients relapsed during biosimilar treatment. Relapse rates and time-to-relapse did not differ between the infliximab originator previously received and biosimilar treatment groups (p = 0.40 and 0.51, respectively). Nine patients experienced 11 adverse events with the infliximab biosimilar, including infections (n = 5), urticaria (n = 4), headache (n = 1), and diarrhea (n = 1). All side effects were grade 2 or less using the WHO classification. CONCLUSIONS: In this retrospective study, the infliximab biosimilar was efficacious and safe for treating Neurosarcoidosis.

  • infliximab biosimilar for treating Neurosarcoidosis tolerance and efficacy in a retrospective study including switch from the originator and initiation of treatment
    Journal of Neurology, 2019
    Co-Authors: Quentin Riller, J. Haroche, Miguel Hie, Camille Cotteret, Helga Junot, Neila Benameur, Makoto Miyara, Patrick Tilleul, A Mathian, Zahir Amoura
    Abstract:

    Infliximab is increasingly used to treat Neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating Neurosarcoidosis. We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in Neurosarcoidosis patients. We compared the survival time without relapse while receiving the biosimilar or previous originator-infliximab treatment. Twenty patients with histologically documented Neurosarcoidosis were treated with an infliximab biosimilar (initiation of treatment in 12 and switch from the originator drug in 8) between February 2016 and August 2018. All patients presenting with neurological involvement of one or more areas, including meningeal (n = 15), cerebral (n = 10), spinal cord (n = 9), and/or cranial nerves (n = 5); epilepsy (n = 3); and/or intracranial hypertension (n = 3) were enrolled. Eighteen patients received glucocorticoids during infliximab treatment, and 16 had methotrexate or azathioprine concomitant treatment. The median duration of follow-up was 25 months (19–28). Six patients relapsed during biosimilar treatment. Relapse rates and time-to-relapse did not differ between the infliximab originator previously received and biosimilar treatment groups (p = 0.40 and 0.51, respectively). Nine patients experienced 11 adverse events with the infliximab biosimilar, including infections (n = 5), urticaria (n = 4), headache (n = 1), and diarrhea (n = 1). All side effects were grade 2 or less using the WHO classification. In this retrospective study, the infliximab biosimilar was efficacious and safe for treating Neurosarcoidosis.

  • The cerebrospinal fluid CD4/CD8 ratio and interleukin‐6 and ‐10 levels in Neurosarcoidosis: a multicenter, pragmatic, comparative study
    European Journal of Neurology, 2019
    Co-Authors: T. Chazal, M. Costopoulos, E. Maillart, C. Fleury, D. Psimaras, P. Legendre, M. Pineton De Chambrun, J. Haroche, Catherine Lubetzki, Zahir Amoura
    Abstract:

    BACKGROUND AND PURPOSE: Neurosarcoidosis is a rare inflammatory disorder of unknown cause. The aim of this study was to evaluate the value of T/B lymphocyte population counts and the concentrations of the cytokines interleukin (IL) 6 and IL-10 in the cerebrospinal fluid (CSF) of Neurosarcoidosis patients. METHODS: A retrospective study CSF biomarkers was conducted in patients with Neurosarcoidosis who underwent CSF analysis between 2012 and 2017 as well as various control populations. RESULTS: Forty-three patients with Neurosarcoidosis, 14 with multiple sclerosis (MS) and 48 with other inflammatory disorders were analyzed. The CSF IL-6 levels were higher in sarcoidosis patients than in MS patients (median 8 vs. 3 pg/ml, P = 0.006). The CSF CD4/CD8 ratio was higher in sarcoidosis patients than in MS patients and in patients with other inflammatory disorders (median 3.18 vs. 2.36 and 2.10, respectively, P = 0.008). The CSF IL-6 level was higher in patients with active Neurosarcoidosis than in non-active Neurosarcoidosis patients (median 13 vs. 3 pg/ml, P = 0.0005). In patients with Neurosarcoidosis, a CSF IL-6 concentration >50 pg/ml was associated with a higher risk of relapse or progression-free survival (hazard ratio 3.60; 95% confidence interval 1.78-23.14). A refractory Neurosarcoidosis patient was treated with an anti-IL-6 monoclonal antibody that produced a complete neurological response. CONCLUSIONS: The CSF CD4/CD8 ratio and IL-6 concentration are increased in Neurosarcoidosis compared to MS and other inflammatory disorders. A CSF IL-6 concentration >50 pg/ml is associated with relapse or progression of Neurosarcoidosis. IL-10 levels may be elevated in Neurosarcoidosis.