Pardal-Fernández José Manuel, Sáez-Méndez Lourdes, Rodríguez-Vázquez María, Godes-Medrano Begoña, Iñíguez-De Onzoño Luis
Servicio de Neurofisiología Clínica, Hospital General Universitario, Albacete, España.
Rev Neurol. 2012 Jan 16;54(2):100-4.
Hypereosinophilic syndrome is produced by what is usually a multiple infiltration of eosinophils into tissues, and may be secondary or idiopathic, depending on whether it is related to a specific aetiology or not. It is not uncommon for it to include nerve disease, but it is unusual for it to do so in the form of multineuritis. Exceptionally, pathogenesis into multiple mononeuritis appears to be related with neurotoxicity due to products derived from eosinophils rather than with infiltrating or inflammatory phenomena. This study describes the case of a female patient with hypereosinophilic syndrome with no verifiable cause, multineuritis and eosinophilic fasciitis.
A 30-year-old female with no relevant history who visited because of some painless inguinal nodules that had appeared several weeks before. At almost the same time, she presented painful sensitive symptoms in her legs with a significant functional incapacity. An important degree of hypereosinophilia, eosinophilic fasciitis and non-neoplastic eosinophilic infiltration of the bone marrow was found, together with multiple mononeuritis. Treatment with oral corticoids improved the dermatological and haematological clinical features, and associating it with gabapentin improved the neuropathic symptoms.
The patient, in accordance with current criteria, presented idiopathic hypereosinophilic syndrome with an undetermined subtype. To our knowledge, the association with eosinophilic fasciitis and multineuritis has not been reported to date. There is no proven infiltrating mechanism in multiple mononeuritis, which corroborates the poor control of the neuropathic clinical symptoms with oral corticoid therapy. Association with gabapentin, which stabilises the axonal membrane, also backs up the neurotoxic pathogenetic hypothesis.
嗜酸性粒细胞增多综合征通常是由嗜酸性粒细胞对组织的多处浸润所致,根据其是否与特定病因相关,可分为继发性或特发性。该综合征合并神经疾病并不少见,但以多发性神经炎形式出现则较为罕见。极少数情况下,多发性单神经炎的发病机制似乎与嗜酸性粒细胞衍生产物的神经毒性有关,而非与浸润或炎症现象有关。本研究描述了一例无明确病因的嗜酸性粒细胞增多综合征女性患者,该患者合并多发性神经炎和嗜酸性筋膜炎。
一名30岁女性,无相关病史,因数周前出现的无痛性腹股沟结节前来就诊。几乎与此同时,她出现了腿部疼痛敏感症状,功能严重受限。检查发现有高度嗜酸性粒细胞增多、嗜酸性筋膜炎以及骨髓非肿瘤性嗜酸性粒细胞浸润,同时伴有多发性单神经炎。口服皮质类固醇治疗改善了皮肤和血液学临床症状,联合加巴喷丁治疗改善了神经病变症状。
根据目前的标准,该患者表现为特发性嗜酸性粒细胞增多综合征,亚型未明。据我们所知,嗜酸性筋膜炎与多发性神经炎的关联此前尚未见报道。多发性单神经炎尚无已证实的浸润机制,这证实了口服皮质类固醇治疗对神经病变临床症状控制不佳。加巴喷丁可稳定轴突膜,其联合使用也支持了神经毒性发病机制假说。