Parra Joana, Domingues Joana, Sargento-Freitas João, Santana Isabel
Department of Neurology, Coimbra Universitary and Hospital Centre, Coimbra, Portugal.
BMJ Case Rep. 2014 Apr 11;2014:bcr2014204130. doi: 10.1136/bcr-2014-204130.
A 56-year-old man presented with weight loss, articular pain and minor neurological symptoms progressing over 1 month. Neurosonological evaluation suggested occlusion in intracranial segments of the left vertebral artery (VA) and of both internal carotid arteries (ICA) and hypoechoic halo sign in both superficial temporal arteries. The diagnosis of giant cell arteritis was supported by inflammatory markers and confirmed by biopsy. Despite early steroid initiation, he manifested fluctuant vascular deficits and became lethargic. Brain MRI indicated watershed infarcts and intracranial dissections of left VA and both ICA. The patient was stabilised with the association of prednisolone 2 mg/kg, methotrexate and oral anticoagulation. Since then he has been neurologically asymptomatic and control imaging showed only residual intracranial left VA stenosis, with no signs of temporal artery inflammation or new vascular lesions. This is to the best of our knowledge, the first reported clinical case with such an extensive intracranial involvement with multiple dissections.
一名56岁男性,出现体重减轻、关节疼痛和轻微神经症状,持续1个月。神经超声检查提示左侧椎动脉(VA)颅内段及双侧颈内动脉(ICA)闭塞,双侧颞浅动脉出现低回声晕征。炎症标志物支持巨细胞动脉炎的诊断,活检予以证实。尽管早期开始使用类固醇治疗,他仍出现波动性血管功能缺损并变得嗜睡。脑部MRI显示分水岭梗死以及左侧VA和双侧ICA的颅内夹层。患者通过联合使用2mg/kg泼尼松龙、甲氨蝶呤和口服抗凝剂得以稳定。从那时起,他在神经方面无症状,对照成像仅显示颅内左侧VA残留狭窄,无颞动脉炎症或新血管病变迹象。据我们所知,这是首例报道的颅内广泛受累伴多发夹层的临床病例。