Freua Fernando, Mahler João Vitor, Lima Pedro Lucas Grangeiro de Sá Barreto, Neville Iuri Santana, Portella Leonardo Barreira, Marussi Victor Hugo Rocha, Lancellotti Carmen Lucia Penteado, Nobrega Paulo Ribeiro, Silva Guilherme Diogo
Beneficência Portuguesa Hospital, São Paulo 01323-001, Brazil.
Clinics Hospital, Faculty of Medicine, University of Sao Paulo, São Paulo 05403-010, Brazil.
Brain Sci. 2023 Aug 11;13(8):1191. doi: 10.3390/brainsci13081191.
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or 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（SLIPPERS）是CLIPPERS谱系的一种罕见变体，迄今为止发表的报告不足十例。关于SLIPPERS本身是否为一种疾病实体，还是仅仅是一个涵盖许多潜在诊断的首字母缩写词，如结节病、血管炎和抗胶质纤维酸性蛋白（GFAP）相关疾病，目前仍在讨论中。一名40岁女性出现语言和注意力障碍发作。磁共振成像（MRI）显示皮质下白质T2/液体衰减反转恢复序列（FLAIR）高信号病变，伴有微结节状、曲线状血管周围对比增强。排除了其他诊断。对类固醇治疗有显著反应。六年后复发，活检显示血管周围T细胞淋巴细胞浸润，无肉芽肿、血管炎或肿瘤形成。类固醇治疗后复发完全缓解。该病例是已报道的诊断为SLIPPERS患者最长的随访记录，6年后的脑活检未提示其他诊断。本报告有助于讨论单纯幕上CLIPPERS样病理可能是一种独立疾病实体的可能性，但需要更多经活检证实且随访时间更长的病例来支持这一假设。最近，GFAP星形细胞病已被明确，可能对应于大量先前诊断为CLIPPERS或SLIPPERS的病例。
