Neurosarcoidosis in a Tertiary Referral Center

Leonhard, Sonja E.; Fritz, Daan; Eftimov, Filip; Kooi, Anneke J. van der; Beek, Diederik van de; Brouwer, Matthijs C.

doi:10.1097/md.0000000000003277

Cited by 57 publications

(85 citation statements)

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“…1), including 1088 patients diagnosed from 1965 to 2015 [6, 8, 9, 11–36]. The number of patients per study ranged from 5 to 305 patients (median 27, interquartile range [IQR] 13–37, Additional file 1: Table S1).…”

Section: Resultsmentioning

confidence: 99%

“…The individual study periods varied between 3 and 31 years (median 13 years, IQR 9–15). Only one of 29 studies was performed prospectively (3%); [6] 24 of 29 (83%) were single-centre studies [6, 9, 11–19, 21–27, 29–31, 34–36]. Inclusion criteria were described in 27 of 29 studies (93%) and were based on the Zajicek criteria in 11 studies [8, 9, 25, 27–31, 33, 35, 36].…”

Section: Resultsmentioning

confidence: 99%

“…Only one of 29 studies was performed prospectively (3%); [6] 24 of 29 (83%) were single-centre studies [6, 9, 11–19, 21–27, 29–31, 34–36]. Inclusion criteria were described in 27 of 29 studies (93%) and were based on the Zajicek criteria in 11 studies [8, 9, 25, 27–31, 33, 35, 36]. Four studies included patients with definite or probable neurosarcoidosis according to these criteria, and seven included definite, probable, or possible neurosarcoidosis [8, 9, 25, 27–31, 33, 35, 36].…”

Section: Resultsmentioning

confidence: 99%

“…Inclusion criteria were described in 27 of 29 studies (93%) and were based on the Zajicek criteria in 11 studies [8, 9, 25, 27–31, 33, 35, 36]. Four studies included patients with definite or probable neurosarcoidosis according to these criteria, and seven included definite, probable, or possible neurosarcoidosis [8, 9, 25, 27–31, 33, 35, 36]. Eleven studies included biopsy-proven sarcoidosis [6, 11, 14, 16, 18, 20–23, 32, 34].…”

Section: Resultsmentioning

confidence: 99%

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Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

2016

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BackgroundNeurosarcoidosis 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.MethodsWe 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.ResultsWe 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%.ConclusionNeurosarcoidosis 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12883-016-0741-x) contains supplementary material, which is available to authorized users.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

2016

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“…[4] However, when sarcoidosis develops exclusively in the nervous system (isolated neurosarcoidosis), diagnosing neurosarcoidosis may be difficult because of the nonspecific clinical features. [5] Furthermore, definite neurosarcoidosis requires histological confirmation, which is often not performed in patients with isolated central nervous system localization of sarcoidosis.…”

Section: Introductionmentioning

confidence: 99%

Isolated neurosarcoidosis mimicking multifocal meningiomas

Wang

et al. 2016

Medicine

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Introduction:Neurosarcoidosis accounts for approximately 5% of the sarcoidosis, which develops exclusively in the nervous system and is always difficult to diagnose. We describe a rare case of isolated neurosarcoidosis mimicking as multifocal meningiomas. A 27-year-old male was admitted to our hospital with a history of unconsciousness and convulsion 1 month ago, which was suspected as a seizure. The results showed no abnormalities in complete blood count; serum electrolytes; erythrocyte sedimentation rate and ultrasonography of the liver, pancreas, spleen, kidney and parotid gland, and so on. Chest radiograph and electroencephalogram were also normal. Serum-angiotensin-converting enzyme slightly increased. Normal opening pressure was shown in cerebrospinal fluid sampling, which includes 8/μL white blood cells, 0.93 g/L protein, and 3.03 mmol/L glucose. Enhanced magnetic resonance imaging revealed multifocal enhancement lesions, including left sphenoid wing region, left temporal and bilateral occipitoparietal region, which were suspected as multiple “meningioma”. A left frontotemporal craniotomy was further performed. Both necrotizing and non-necrotizing granulomas were revealed in the pathological specimen, most of which were associated with multinucleated giant cells and macrophages. We could also see the fibrosis and inflammatory reaction in the sample composed of lymphocytes, histiocytes, and plasma cells. Histopathological examination showed that the cells were positive for human CD68 (KP1), CD68 (PGM1), and CD163; however, they were negative for the AF, epithelial membrane antigen, and glial fibrillary acidic protein. Tuberculosis-deoxyribonucleic acid test and special stains for acid-fast bacilli and fungi were negative. The diagnosis was finally made as isolated neurosarcoidosis. Then the patient was treated with additional corticosteroid therapy. Serial imaging examination 4 months later revealed that the lesions extremely decreased.Conclusion:The diagnosis of isolated central nervous system sarcoidosis was still difficult because of limitations of available diagnostic tests. So neurosarcoidosis in the clinical work should never be neglected.

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Definition and Consensus Diagnostic Criteria for Neurosarcoidosis

et al. 2018

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ypical presentations of sarcoidosis permit ready diagnosis in a substantial proportion of individuals with this disease; however, the diagnosis is often rendered difficult by unusual clinical manifestations or diagnostic mimics, which can be organ specific. This difficulty is particularly evident in neurosarcoidosis (NS). 1 Despite numerous publications on NS, no consensus definition of the entity exists and no consensus perspective addresses the prerequisites for establishing a diagnosis. Development of criteria for diagnosis is essential for the design of clinical trials for NS and is valuable to the patient and clinician. A consortium of physicians with expertise in NS has developed consensus criteria for the diagnosis of NS. Because sarcoidosis can affect the central nervous system (CNS) and peripheral nervous system (PNS), occasionally in the absence of other organ involvement, we propose harmonized diagnostic criteria for both the CNS and PNS. MethodsThe Neurosarcoidosis Consortium Consensus Group consisted of 14 members: 10 neurologists and 4 pulmonologists. The group met 5 times in person and conducted additional work via electronic communication. The group started with the premise that a need existed for consensus diagnostic criteria for NS to improve clinical care and support research. The group agreed that diagnostic labels about NS should be based on level of certainty and provide specific guid-IMPORTANCE The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease.OBSERVATIONS The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis.CONCLUSIONS AND RELEVANCE Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue...

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Neurosarcoidosis in a Tertiary Referral Center

Abstract: Supplemental Digital Content is available in the text

Cited by 57 publications

References 25 publications

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

Isolated neurosarcoidosis mimicking multifocal meningiomas

Definition and Consensus Diagnostic Criteria for Neurosarcoidosis

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