与可能的颅内血管炎性动脉病相关的Collet-Sicard综合征：一例病例报告

文献信息

中文摘要

背景： Collet-Sicard综合征是舌咽神经、迷走神经、副神经和舌下神经的单侧麻痹。它最常由颅底的肿瘤性、创伤性或血管性病变引起。据我们所知，其与颅内血管炎的关联此前尚未被报道。
病例介绍： 一名35岁斯里兰卡女性因3个月吞咽困难、发声困难和鼻音性构音障碍病史就诊。她1年前曾发生左侧脑梗死。检查显示右侧第IX、X、XI和XII脑神经麻痹，符合Collet-Sicard综合征。颅底增强计算机断层扫描和磁共振成像正常，无颈静脉孔病变、颅底骨髓炎、静脉血栓形成或基底脑膜炎的证据。脑脊液分析显示轻度淋巴细胞增多和蛋白升高。广泛检查排除了恶性肿瘤、感染、结节病、IgG4相关疾病和系统性血管炎。对既往神经影像的回顾显示胼胝体梗死。由于这一不寻常的卒中部位，进行了数字减影血管造影，显示多灶性颅内动脉狭窄和管腔不规则，提示可能的颅内血管炎。在缺乏其他解释的情况下，我们推测下组脑神经病变可能源于血管炎累及神经滋养血管。她接受了皮质类固醇、利妥昔单抗、硫唑嘌呤和阿司匹林治疗。尽管接受了治疗，严重的延髓功能障碍仍持续存在，她后来死于吸入性肺炎。
结论： 本病例突出了Collet-Sicard综合征与颅内血管炎性动脉病之间的一种罕见关联。当不存在结构性颅底病变时，应将血管炎性神经病视为多发性下组脑神经病变的潜在原因。胼胝体梗死是一种不常见的卒中模式，应促使对包括中枢神经系统血管炎在内的不寻常血管病因进行检查。

英文摘要

BACKGROUND: Collet-Sicard syndrome is unilateral palsy of the glossopharyngeal, vagus, accessory, and hypoglossal nerves. It is most commonly caused by neoplastic, traumatic, or vascular lesions at the skull base. To our knowledge, its association with intracranial vasculitis has not been reported previously.
CASE PRESENTATION: A 35-year-old Sri Lankan woman presented with a 3-month history of dysphagia, dysphonia, and nasal dysarthria. She had had a left-sided cerebral infarction 1 year earlier. Examination showed right-sided cranial nerves IX, X, XI, and XII palsies, consistent with Collet-Sicard syndrome. Contrast-enhanced computed tomography and magnetic resonance imaging of the skull base were normal, with no evidence of jugular foramen pathology, skull-base osteomyelitis, venous thrombosis, or basal meningitis. Cerebrospinal fluid analysis revealed mild lymphocytic pleocytosis and elevated protein. Extensive investigations excluded malignancy, infection, sarcoidosis, IgG4-related disease, and systemic vasculitis. Review of prior neuroimaging showed a corpus callosal infarction. Because of this unusual stroke location, digital subtraction angiography was performed which demonstrated multifocal intracranial arterial stenoses and luminal irregularities suggestive of possible intracranial vasculitis. In the absence of another explanation, we hypothesised that the lower cranial neuropathies may have resulted from vasculitic involvement of the vasa nervorum. She was treated with corticosteroids, rituximab, azathioprine, and aspirin. Despite treatment, severe bulbar dysfunction persisted, and she later died from aspiration pneumonia.
CONCLUSIONS: This case highlights a rare association between Collet-Sicard syndrome and intracranial vasculitic arteriopathy. When structural skull-base lesions are absent, vasculitic neuropathy should be considered as a potential cause of multiple lower cranial neuropathies. Corpus callosal infarction, an uncommon stroke pattern, should prompt investigation for unusual vascular aetiologies including central nervous system vasculitis.