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Neurosarcoidosis: clinical characteristics, diagnosis, and treatment in eight Chinese patients

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Abstract

Neurosarcoidosis is relatively rare and has diverse manifestations. The clinical characteristics, diagnosis, treatment, and outcome for neurosarcoidosis in China are poorly understood. We retrospectively analyzed the clinical features, laboratory and imaging results, treatment, and outcomes in patients who met the criteria for definite or probable neurosarcoidosis in Xuan Wu Hospital of Capital Medical University from 2000 to 2015. Eight patients were included in this study, accounting for 5.84% of all cases with sarcoidosis. The mean age at onset was 50.25 years, and 75% of the patients were female. Five cases had a prior diagnosis of extraneurologic sarcoidosis, leading to a shorter lag time between onset of symptoms and diagnosis (3.4 vs. 16.2 months). Neurological symptoms were the first clinical feature of sarcoidosis in three cases, and no patients presented isolated nervous system manifestation. The most common symptom was sensory disturbance, and the most common site of nervous system involvement was brain parenchyma and meninges. Disturbance of consciousness, seizures, hydrocephalus, and abnormal CSF assays were associated with poor prognosis. All patients were treated with corticosteroids and one was also given azathioprine. Five patients had complete or partial improvement, one remained stabilized, and two deteriorated and died. Neurosarcoidosis is difficult to diagnose early and might be associated with a poor prognosis. Tissue biopsy for a definitive diagnosis and aggressive therapy with corticosteroids plus other alternative immunosuppressive treatment should be recommended in China.

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Acknowledgments

This work was supported by the grants from the National Natural Science Foundation of China (No. 81100797) and Beijing High-Level Health Talents Training Project (No. 2015-3-068).

Funding

This work was supported by the grants from the National Natural Science Foundation of China (No. 81100797) and Beijing High-Level Health Talents Training Project (No. 2015–3-068).

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Authors and Affiliations

  1. Department of Neurology, Xuan Wu Hospital, Capital Medical University, Changchun Street 45, Xicheng District, Beijing, 100053, People’s Republic of China

    Fen Wang, Dongmei Guo, Zheng Liu, Aihong Zhou, Cuibai Wei & Jianping Jia

Authors
  1. Fen Wang
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  2. Dongmei Guo
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  3. Zheng Liu
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  4. Aihong Zhou
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  5. Cuibai Wei
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  6. Jianping Jia
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Contributions

FW, DG, and JJ contributed to the study design, analysis, and interpretation of the data. FW, ZL, AZ, and CW contributed to the acquisition of data. FW contributed to drafting the manuscript for content. All the authors (FW, DG, ZL, AZ, CW, and JJ) contributed to revising the manuscript. JJ and FW conducted the study coordination. All the authors gave final approval of the submitted version.

Corresponding author

Correspondence to Jianping Jia.

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Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical or institutional review board approval

This retrospective study received ethical approval from the Xuan Wu Hospital institutional review board. Formal consent is not required for this type of study.

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Wang, F., Guo, D., Liu, Z. et al. Neurosarcoidosis: clinical characteristics, diagnosis, and treatment in eight Chinese patients. Neurol Sci 39, 1725–1733 (2018). https://doi.org/10.1007/s10072-018-3491-2

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  • DOI: https://doi.org/10.1007/s10072-018-3491-2

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