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首页> 外文期刊>The neurologist. >Vasculitis Presenting as Autonomic Failure
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Vasculitis Presenting as Autonomic Failure

机译：血管炎作为自主失败

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Introduction: Granulomatosis with polyangiitis (GPA) is a vasculitic process that can cause neurological dysfunction in addition to characteristic sinus and pulmonary manifestations. This case report highlights the spectrum of nervous system manifestations and includes rarely reported autonomic and pituitary involvement. Case Report: A 62-year-old woman presented with orthostatic intolerance, tachycardia, dry mouth, and temperature sensitivity; subsequent autonomic reflex study demonstrated widespread postganglionic sympathetic sudomotor, cardiovagal, and cardiovascular adrenergic impairment reflective of autonomic neuropathy and overall autonomic failure. Additional progressive symptoms included dysarthria, dysphagia, bilateral hearing loss, voice hoarseness, and right-sided facial numbness with multiple cranial neuropathies identified on neurological examination. The diagnosis of central diabetes insipidus was also confirmed. Pachymeningitis was present on brain magnetic resonance imaging. Pathologic review of the dural biopsy specimen revealed necrotizing granulomatous vasculitis consistent with GPA. She was treated with intravenous methylprednisolone and rituximab. Over the next 2 months, she had near-complete resolution of her symptoms with normalization on repeat autonomic testing. Conclusions: This is a unique GPA case presenting with autonomic failure and pituitary dysfunction with conclusive objective findings of autonomic dysfunction. Autonomic dysfunction and other disease manifestations were responsive to immunosuppressive therapy.
机译：简介：肉芽肿伴多血管炎（GPA）是一种血管炎过程，除了特征性的窦道和肺部表现外，还可导致神经功能障碍。本病例报告强调了神经系统表现的范围，包括罕见的自主神经和垂体受累。病例报告：一名62岁女性，表现为立位不耐受、心动过速、口干和温度敏感性；随后的自主神经反射研究显示，广泛的节后交感汗运动、心脏迷走神经和心血管肾上腺素能损伤反映了自主神经病变和整体自主神经功能衰竭。其他进行性症状包括构音障碍、吞咽困难、双侧听力丧失、声音嘶哑和右侧面部麻木，神经系统检查发现多发性颅神经病变。中枢性尿崩症的诊断也得到了证实。脑磁共振成像显示有硬脑膜炎。硬脑膜活检标本的病理检查显示坏死性肉芽肿性血管炎符合GPA。她接受了静脉注射甲基强的松龙和利妥昔单抗的治疗。在接下来的两个月里，她的症状几乎完全缓解，通过反复自主神经测试恢复正常。结论：这是一个独特的GPA病例，表现为自主神经功能衰竭和垂体功能障碍，并有明确的自主神经功能障碍客观结果。自主神经功能障碍和其他疾病表现对免疫抑制治疗有反应。

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