Prickett Joshua, Altafulla Juan, Knisely Anna, Litvack Zachary
Department of Skull Base and Minimally Invasive Neurosurgery, Swedish Neuroscience Institute, Seattle, Washington, United States.
Department of Neuroanatomy, Seattle Science Foundation, Seattle, Washington, United States.
J Neurol Surg Rep. 2019 Jul;80(3):e31-e35. doi: 10.1055/s-0039-1687886. Epub 2019 Jul 18.
Encephalitis due to has been seen almost exclusively in patients with severe compromise of their immune systems, such as acquired immune deficiency syndrome (AIDS). Fungal sinusitis with frank invasion through the cranial base and subsequent seeding of the central nervous system is rare, but should be considered in the differential of patients presenting with meningitis and sinus/skull base lesions even without obvious immune compromise. Improvements in diagnostic testing has increased the ability to correctly identify and new antimicrobials have allowed a condition that once carried a high morbidity and mortality to be managed with better outcomes. We present our treatment algorithm for successful management of an immunocompetent patient with extensive fungal encephalitis due to erosion through the skull base. The patient is a 59-year-old male presenting unresponsive with sphenoid mass erosive of the skull base and symptoms of meningitis and encephalitis due to . Magnetic resonance imaging (MRI) at presentation demonstrated extensive diencephalic invasion, and a sphenoid mass with erosion of the skull base. Lumbar puncture (LP) confirmed elevated opening pressure of 45 cm H2O, and cultures confirmed infection with . He underwent operative sinonasal debridement followed by placement of an external ventricular drain for management of hydrocephalus. He was treated aggressively with a combination of both intravenous (IV) amphotericin B daily and intrathecal amphotericin B via the ventriculostomy thrice weekly. By the 2nd week of treatment, patient regained consciousness. After 4 weeks of therapy, cerebrospinal fluid (CSF) cultures turned negative, and the external ventricular drain (EVD) was converted to a ventriculoperitoneal shunt (VPS) to manage chronic postinfectious hydrocephalus. We also placed a contralateral Ommaya reservoir to permit continued weekly intrathecal amphotericin B without violation of the shunt valve. With each instillation, the shunt was set to its highest setting to minimize CSF egress for 6 hours then reset to its "drainage" setting. After an additional 6 weeks of outpatient therapy, intrathecal therapy was discontinued. We continued CSF surveillance via Ommaya sampling monthly. At 9-month follow-up, he has remained clinically stable without evidence of recurrent infection. He has residual mild cognitive deficits, but is living semiindependently with his brother. Fungal sinusitis is uncommon, especially in those without significantly compromised immune systems. Invasive fungal meningitis resulting in meningitis and encephalitis is even rarer. The condition carries high morbidity and mortality that can only be mitigated with a multidisciplinary effort by neurosurgery, otolaryngology, and infectious disease specialists. While there are no clear treatment guidelines, we present an approach that may permit longer term independent survival.
由[具体真菌名称未给出]引起的脑炎几乎仅见于免疫系统严重受损的患者,如获得性免疫缺陷综合征(艾滋病)患者。真菌性鼻窦炎伴颅骨基部的明显侵袭及随后的中枢神经系统播散很少见,但即使在没有明显免疫功能低下的情况下,对于出现脑膜炎和鼻窦/颅底病变的患者进行鉴别诊断时也应考虑到。诊断检测方法的改进提高了正确识别的能力,新的抗菌药物使得一种曾经具有高发病率和死亡率的疾病能够得到更好的治疗。我们展示了我们对于一名免疫功能正常但因颅骨基部侵蚀而患有广泛真菌性脑炎患者的成功治疗方案。
该患者为一名59岁男性,呈无反应状态,伴有侵蚀颅骨基部的蝶窦肿物以及由[具体真菌名称未给出]引起的脑膜炎和脑炎症状。就诊时的磁共振成像(MRI)显示间脑广泛受侵,以及一个侵蚀颅骨基部的蝶窦肿物。腰椎穿刺(LP)证实初压升高至45 cm H₂O,培养证实感染[具体真菌名称未给出]。他接受了鼻窦清创手术,随后放置了外部脑室引流管以治疗脑积水。他接受了积极治疗,每日静脉注射两性霉素B,并通过脑室造瘘每周三次鞘内注射两性霉素B。治疗第2周时,患者恢复意识。治疗4周后,脑脊液(CSF)培养转阴,外部脑室引流管(EVD)转换为脑室腹腔分流术(VPS)以治疗慢性感染后脑积水。我们还放置了对侧的Ommaya储液器,以便在不破坏分流阀的情况下继续每周进行鞘内注射两性霉素B。每次注射时,将分流器设置到最高档位,使脑脊液流出最少6小时,然后再设置回“引流”档位。在门诊继续治疗6周后,停止鞘内治疗。我们通过每月一次的Ommaya采样继续进行脑脊液监测。在9个月的随访中,他临床状况稳定,没有复发感染的迹象。他有轻度的认知功能缺损残留,但与他的兄弟半独立生活。
真菌性鼻窦炎并不常见,尤其是在那些免疫功能没有明显受损的患者中。导致脑膜炎和脑炎的侵袭性真菌性脑膜炎更为罕见。这种疾病具有高发病率和死亡率,只有通过神经外科、耳鼻喉科和传染病专家的多学科努力才能缓解。虽然没有明确的治疗指南,但我们展示了一种可能允许患者长期独立生存的治疗方法。