Tsuboyama Melissa, Chandler Justin V, Scharf Eugene, Scagnelli John, Ramchandran Rajeev S, Holloway Robert, Wychowski Thomas
Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA.
Raleigh Neurology Associates, Raleigh, NC, USA.
Neurohospitalist. 2018 Jul;8(3):146-151. doi: 10.1177/1941874417749379. Epub 2018 Jan 17.
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a self-limited idiopathic inflammatory ophthalmologic condition with characteristic funduscopic and fluorescein angiography findings. It is typically characterized by a flu-like prodrome followed by monocular or binocular vision loss. Often, prognosis is excellent with complete or near-complete recovery of vision. Rarely, however, APMPPE is associated with neurologic complications, including meningitis, cerebral vasculitis, and stroke. Treatment in patients with central nervous system (CNS) involvement involves steroids and ultimately other immunosuppressive therapy, as there can be significant resulting morbidity and mortality otherwise. Evidence or guidelines regarding duration of treatment are lacking. We present 4 patients diagnosed with APMPPE who demonstrate the spectrum of neurologic sequelae associated with APMPPE. The first 2 cases highlight cerebrospinal fluid lymphocytic pleocytosis as an indicator of active CNS inflammation and the potential utility of serial lumbar punctures (LPs) to guide treatment duration. Cases 3 and 4 demonstrate the neurovascular complications seen in CNS vasculitis. Case 4 also highlights the potential use of magnetic resonance vessel wall imaging (VWI) as a noninvasive means for disease surveillance and treatment guidance. This case series emphasizes the importance of recognition by neurologists of APMPPE as an entity associated with strokes and cerebral vasculitis in order to provide appropriate and timely treatment. Active CNS inflammation warrants continued aggressive immunosuppressant treatment, and we propose that serial LPs and/or magnetic resonance VWIs may be effective tools to guide disease surveillance and subsequent treatment duration.
急性后极部多灶性鳞状色素上皮病变（APMPPE）是一种自限性特发性炎症性眼科疾病，具有特征性的眼底和荧光素血管造影表现。其典型特征为类似流感的前驱症状，随后出现单眼或双眼视力丧失。通常，视力预后良好，可完全或近乎完全恢复。然而，APMPPE很少与神经系统并发症相关，包括脑膜炎、脑血管炎和中风。中枢神经系统（CNS）受累患者的治疗包括使用类固醇，最终可能需要其他免疫抑制治疗，否则可能会导致严重的发病率和死亡率。目前缺乏关于治疗持续时间的证据或指南。我们报告了4例被诊断为APMPPE的患者，他们展示了与APMPPE相关的一系列神经系统后遗症。前2例突出了脑脊液淋巴细胞增多作为中枢神经系统活动性炎症的指标以及连续腰椎穿刺（LP）在指导治疗持续时间方面的潜在作用。第3例和第4例展示了中枢神经系统血管炎中出现的神经血管并发症。第4例还强调了磁共振血管壁成像（VWI）作为疾病监测和治疗指导的非侵入性手段的潜在用途。这个病例系列强调了神经科医生认识到APMPPE是一种与中风和脑血管炎相关的疾病实体的重要性，以便提供适当和及时的治疗。中枢神经系统活动性炎症需要持续积极的免疫抑制治疗，我们建议连续腰椎穿刺和/或磁共振血管壁成像可能是指导疾病监测和后续治疗持续时间的有效工具。
