Russo Mirella, Piermartiri Alberto, Carrarini Claudia, Dono Fedele, De Angelis Maria Vittoria, Sensi Stefano L, Onofrj Marco, Bonanni Laura
Department of Neurosciences, Imaging and Clinical Sciences, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy.
Behavioral Neurology and Molecular Neurology Units, Center of Excellence on Aging and Translational Medicine (CeSI-MeT), University G. d'Annunzio of Chieti-Pescara, Chieti, Italy.
Case Rep Neurol. 2020 Sep 18;12(3):307-313. doi: 10.1159/000508945. eCollection 2020 Sep-Dec.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder featured by pontocerebellar dysfunctions and, in some cases, later cognitive disturbances. Here, we describe an atypical presentation of CLIPPERS, characterized by clinical onset with neuropsychiatric and cognitive symptoms. A 45-year-old man was referred to our Memory Clinic due to difficulties at work for over a month, caused by confusion and asthenia. Furthermore, insomnia and mood changes appeared. These disturbances were unresponsive to antipsychotic and antidepressant drugs. At admission, the patient presented also with severe cognitive impairment, urinary incontinence, ataxic gait, and limitation of lateral conjugate gaze. During the hospitalization, the patient underwent cerebrospinal fluid analysis, serum systemic autoimmune disorders laboratory research, neoplastic markers analysis, and brain MRI scan. The radiological and laboratory findings were compatible with the diagnosis of CLIPPERS. The sudden clinical and radiological improvement of the patient's conditions, after only a week of steroid therapy, further confirmed our clinical suspicion. The present case enhances the necessity to consider CLIPPERS in the differential diagnosis of pre-senile cognitive impairment, even in the absence of early pontocerebellar neurological signs. Before the spreading of the neuroinflammatory and degenerative processes, CLIPPERS represents one among the few possible reversible causes of cognitive decline.
类固醇反应性桥脑周围血管强化的慢性淋巴细胞性炎症（CLIPPERS）是一种罕见的炎症性疾病，其特征为桥小脑功能障碍，部分病例后期出现认知障碍。在此，我们描述一例CLIPPERS的非典型表现，其临床起病以神经精神和认知症状为特征。一名45岁男性因工作困难一个多月前来我们的记忆门诊就诊，工作困难由意识模糊和乏力引起。此外，还出现了失眠和情绪变化。这些障碍对抗精神病药物和抗抑郁药物均无反应。入院时，患者还存在严重认知障碍、尿失禁、共济失调步态以及侧方共轭凝视受限。住院期间，患者接受了脑脊液分析、血清系统性自身免疫性疾病实验室检查、肿瘤标志物分析以及脑部MRI扫描。影像学和实验室检查结果与CLIPPERS的诊断相符。仅经过一周的类固醇治疗，患者的病情便在临床和影像学上突然改善，这进一步证实了我们的临床怀疑。本病例强调了即使在没有早期桥小脑神经体征的情况下，在老年前期认知障碍的鉴别诊断中考虑CLIPPERS的必要性。在神经炎症和退行性过程扩散之前，CLIPPERS是认知衰退少数可能的可逆原因之一。
