Challa Anusha, Sirisha Sai, Khandelia Harsh, Parekh Mihir, Patil Anuja, Jayalakshmi Sita
Department of Neurology, Krishna Institute of Medical Sciences, Secunderabad, India.
Department of Critical Medicine, Krishna Institute of Medical Sciences, Secunderabad, India.
Oxf Med Case Reports. 2023 Jul 18;2023(7):omad067. doi: 10.1093/omcr/omad067. eCollection 2023 Jul.
Eosinophilic granulomatous polyangiitis (EGPA) like other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has multisystemic involvement. It commonly manifests with prodromal pulmonary involvement as asthma, chronic sinusitis followed by systemic vasculitic complications associated with blood and tissue eosinophilia. Central nervous system manifestations at presentation are uncommon compared with peripheral nervous system involvement. Vasculitic neuropathy in EGPA commonly presents as mononeuritis multiplex but rarely as polyradiculopathy. Late onset EGPA often presents with systemic involvement, and early diagnosis is a key to prevent further complications. The neuropathy in late onset EGPA is often refractory to immunosuppression and corticosteroids treatment. We report a case of EGPA with late onset asthma presenting with acute infarct and demyelinating polyradiculoneuropathy that progressed with bulbar paralysis and profound dysautonomia. This illustrates simultaneous involvement of central and peripheral nervous system with EGPA. Autonomic dysfunction can occur in patients of EGPA with multisystem involvement, which may predict severe complications.
嗜酸性肉芽肿性多血管炎(EGPA)与其他抗中性粒细胞胞浆抗体(ANCA)相关血管炎一样,具有多系统受累的特点。它通常先出现前驱性肺部受累,表现为哮喘、慢性鼻窦炎,随后出现与血液和组织嗜酸性粒细胞增多相关的系统性血管炎并发症。与周围神经系统受累相比,EGPA患者就诊时出现中枢神经系统表现并不常见。EGPA中的血管炎性神经病变通常表现为多发性单神经炎,但很少表现为多发性神经根病。迟发性EGPA常表现为系统性受累,早期诊断是预防进一步并发症的关键。迟发性EGPA中的神经病变通常对免疫抑制和皮质类固醇治疗无效。我们报告一例迟发性哮喘合并EGPA的病例,该患者出现急性梗死和脱髓鞘性多发性神经根神经病,并进展为延髓麻痹和严重自主神经功能障碍。这说明了EGPA同时累及中枢和周围神经系统。自主神经功能障碍可发生在多系统受累的EGPA患者中,这可能预示着严重并发症。