摘要：

目的 总结累及脑神经的鼻脑型毛霉菌病的临床特征、诊断与治疗经过、转归。方法与结果 纳入2012年2月至2020年1月在首都医科大学附属北京同仁医院经活检病理证实的8例累及脑神经的鼻脑型毛霉菌病患者，临床主要表现为头痛、眼周和面部肿胀；除累及副鼻窦外，还累及眼动神经、眼外肌、脑膜、脑实质、视神经、脑血管和前庭蜗神经。原发病分别为2型糖尿病（5例），急性髓细胞性白血病（1例）、重症再生障碍性贫血（1例）和颅脑创伤术后（1例）；6例行病变组织黏膜活检术，1例行脑实质活检术，1例行颞部肌肉组织活检术。除1例放弃治疗死亡；其余7例均接受两性霉素B或两性霉素B脂质复合体抗真菌治疗，其中1例联合泊沙康唑、4例同时行经鼻内镜根治性病变清除术，3例累及脑实质者死亡。结论 累及脑实质的鼻脑型毛霉菌病病死率极高，对于存在免疫功能低下的鼻窦炎患者，若出现急性进展的眼动障碍、眼周肿胀和头痛，应注意鼻脑型毛霉菌病的可能，尽早予抗真菌药物联合手术治疗，病变部位黏膜组织活检术有助于快速诊断。

关键词: 毛霉菌病, 鼻疾病, 脑疾病, 颅神经, 活组织检查

Abstract:

Objective To summarize the clinical features and rhinocerebral mucormycosis involving cranial nerve. Methods and Results Eight pathologic confirmed cases of rhinocerebral mucormycosis with cranial nerves involvement since February 2012 to January 2020 were collected and evaluated. All patients were diagnosed at Beijing Tongren Hospital, Capital Medical University. The main clinical manifestations were headache, eye and face swelling. In addition to the paranasal sinuses, the ocular motor nerve, extraocular muscle, meninges, brain parenchyma, optic nerve, cerebrovascular and vestibulocochlear nerve were also involved. There were 5 patients with the primary disease of type 2 diabetes mellitus, one patient with acute leukemia receiving chemotherapy, one patient with severe aplastic anemia receiving immunosuppressive therapy, and one patient with craniocerebral trauma. Six patients were confirmed by pathological biopsy of paranasal sinus mucosa, the other 2 patients underwent brain parenchymal biopsy and temporal muscle biopsy respectively. In addition to one patient with meningeal involvement who gave up treatment and finally died, the other 7 patients received amphotericin B (one case was combined with posaconazole) or amphotericin B liposome antifungal therapy. Among them 4 patients underwent simultaneous transnasal endoscopic radical lesion removal treatment and 3 patients with brain parenchymal involvement died despite of active treatment. Conclusions The mortality of rhinocerebral mucormycosis with brain parenchyma involvement is extremely high. For patients with sinusitis with immunocompromised state, if there is acute progression of cranial nerve involvement, swelling around the eyes and headache, attention should be paid to the identification of the disease, and antifungal drugs and combined surgery treatment should be given early. Pathological biopsy of the affected mucosal tissue is the first choice for quick diagnosis.

Key words: Mucormycosis, Nose diseases, Brain diseases, Cranial nerves, Biopsy