孤立性疼痛性三叉神经神经病作为类固醇反应性伴有桥脑血管周围强化的慢性淋巴细胞炎症的不常见表现：一例报告。
Isolated painful trigeminal neuropathy as an unusual presentation of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A case report.
机构信息
1 Department of Ophthalmology, Westmead Hospital, Sydney, Australia.
2 Prince of Wales Clinical School, University of New South Wales, Sydney, Australia.
出版信息
Cephalalgia. 2019 Feb;39(2):316-322. doi: 10.1177/0333102418781789. Epub 2018 Jun 4.
BACKGROUND
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory central nervous system disorder, typically presenting with subacute symptoms referable to brainstem and cerebellar pathology. This is the first report of CLIPPERS presenting with a painful trigeminal neuropathy.
CASE REPORT
We report an unusual case of CLIPPERS presenting with facial pain and sensory symptoms, in the absence of other brainstem or cerebellar signs. Perivascular enhancement of peri-pontine structures on neuroimaging, lymphocytic infiltrate on histopathology and rapid clinical and radiological responsiveness to glucocorticosteroids were key to diagnosis. Extensive investigations excluded various differential aetiologies.
CONCLUSION
The pathogenesis of CLIPPERS is poorly understood, and the diagnostic criteria are yet to be validated. In this case, facial pain was not associated with other brainstem or cerebellar signs, broadening current understanding of how CLIPPERS may present. This has clinical implications in guiding future investigations for patients presenting with painful trigeminal neuropathy.
背景
伴有桥脑旁血管周围强化对类固醇有反应的慢性淋巴细胞炎症（CLIPPERS）是一种中枢神经系统炎症性疾病，通常表现为亚急性脑干和小脑病变的症状。这是首例表现为三叉神经痛的 CLIPPERS 病例报告。
病例报告
我们报告了一例不常见的 CLIPPERS 病例，其表现为面部疼痛和感觉症状，而无其他脑干或小脑体征。神经影像学上桥旁结构的血管周围增强、组织病理学上的淋巴细胞浸润以及对糖皮质激素的快速临床和放射学反应是诊断的关键。广泛的检查排除了各种不同的病因。
结论
CLIPPERS 的发病机制尚不清楚，诊断标准仍有待验证。在本例中，面部疼痛与其他脑干或小脑体征无关，拓宽了当前对 CLIPPERS 表现方式的认识。这对指导有三叉神经痛的患者进行未来的检查具有临床意义。
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