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SLIPPERS综合征的诊断挑战:病例报告

Diagnostic challenges in SLIPPERS syndrome: Case report.

作者信息

Acır İbrahim, Sezen Ahmetcan, Aygün Murat Serhat, Altıntaş Ayşe

机构信息

Bakırköy Dr Sadi Konuk Training and Research Hospital, Department of Neurology, Istanbul/Türkiye.

Koç University, Faculty of Medicine, Istanbul/Türkiye.

出版信息

Mult Scler J Exp Transl Clin. 2025 Jan 31;11(1):20552173241312534. doi: 10.1177/20552173241312534. eCollection 2025 Jan-Mar.

DOI:10.1177/20552173241312534
PMID:39895881
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11783460/
Abstract

BACKGROUND

SLIPPERS (Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids) is a rare variant of a syndrome called CLIPPERS (Chronic Lymphocytic Inflammation with Ponsine Perivascular Enhancement Responsive to Steroids). SLIPPERS is characterized by distinct supratentorial lesions that share radiological and pathological characteristics with CLIPPERS. The ongoing issue is whether these syndromes should be considered as a distinct disease entity or simply a form for a variety of underlying conditions such as granulomatosis, vasculitis, and infectious diseases.

CASE

We present a unique case of SLIPPERS observed in a 26-year-old woman with no notable medical or familial background. Laboratory findings ruled out certain diseases from the list of differentials and cranial MRI showed T2 hyperintense areas with linear-patchy enhancements, a pattern consistent with SLIPPERS syndrome. Consequently, patient was diagnosed with SLIPPERS syndrome and received methylprednisolone therapy.

CONCLUSION

Both SLIPPERS and CLIPPERS are complicated syndromes posing diagnostic challenges and requiring careful investigation to avoid misdiagnosis. Following a thorough differential diagnosis, appropriate treatment can be initiated, and follow-up is required.

摘要

背景

SLIPPERS(幕上淋巴细胞性炎症伴实质血管周围强化且对类固醇有反应)是一种名为CLIPPERS(慢性淋巴细胞性炎症伴脑桥血管周围强化且对类固醇有反应)综合征的罕见变体。SLIPPERS的特征是独特的幕上病变,这些病变与CLIPPERS具有相同的放射学和病理学特征。目前的问题是,这些综合征应被视为一种独特的疾病实体,还是仅仅是各种潜在疾病(如肉芽肿病、血管炎和传染病)的一种形式。

病例

我们报告了一例在一名无明显病史或家族史的26岁女性中观察到的SLIPPERS独特病例。实验室检查结果排除了鉴别诊断清单中的某些疾病,头颅磁共振成像显示T2高信号区伴有线性斑片状强化,这一模式与SLIPPERS综合征一致。因此,该患者被诊断为SLIPPERS综合征并接受了甲泼尼龙治疗。

结论

SLIPPERS和CLIPPERS都是复杂的综合征,带来诊断挑战,需要仔细检查以避免误诊。经过全面的鉴别诊断后,可以开始适当的治疗,并需要进行随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/79aa92292fc9/10.1177_20552173241312534-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/52658c65f5d2/10.1177_20552173241312534-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/7747756556e2/10.1177_20552173241312534-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/79aa92292fc9/10.1177_20552173241312534-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/52658c65f5d2/10.1177_20552173241312534-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/7747756556e2/10.1177_20552173241312534-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa87/11783460/79aa92292fc9/10.1177_20552173241312534-fig3.jpg

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本文引用的文献

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Orphanet J Rare Dis. 2024 Aug 14;19(1):297. doi: 10.1186/s13023-024-03319-2.
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Unusual CLIPPERS presentation with a predominant spinal cord involvement: case report and review of the literature.伴有明显脊髓受累的不典型 CLIPPERS 表现:病例报告及文献复习。
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Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)-Does it Really Exist?
对类固醇有反应的幕上淋巴细胞性炎症伴实质血管周围强化(SLIPPERS)——它真的存在吗?
Brain Sci. 2023 Aug 11;13(8):1191. doi: 10.3390/brainsci13081191.
4
The Elusive SLIPPERS Syndrome (Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids): A Case Report and Literature Review.难以捉摸的SLIPPERS综合征(幕上淋巴细胞性炎症伴实质血管周围强化且对类固醇有反应):病例报告及文献综述
Int Med Case Rep J. 2023 Jun 10;16:357-361. doi: 10.2147/IMCRJ.S411204. eCollection 2023.
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Seronegative autoimmune diseases: A challenging diagnosis.血清阴性自身免疫性疾病:诊断难题。
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Case 302: Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids.病例 302:伴有实质血管周围强化的幕上淋巴细胞炎症,对类固醇有反应。
Radiology. 2022 Jun;303(3):726-730. doi: 10.1148/radiol.204423.
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MRI findings of autoimmune glial fibrillary acidic protein astrocytopathy involving infratentorial: Case report.幕下受累的自身免疫性胶质纤维酸性蛋白星形细胞病的MRI表现:病例报告
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