Neurosurgery Department, Kaiser Sacramento, 2025 Morse Avenue, Sacramento, CA, 95825, USA.
Neurocrit Care. 2019 Apr;30(2):272-279. doi: 10.1007/s12028-018-0538-4.
Initially reported in tropical regions, Cryptococcus gattii infection is now diagnosed globally. Methods: case report; Literature review. Although initial reports described outbreaks of pulmonary and central nervous system (CNS) disease in tropical regions such as Australia and New Guinea, it is now clear that Cryptococcus gattii is a global, neurotropic pathogen. In contrast with C. neoformans, C. gattii patients are more likely to present with cryptococcomas in the brain and lungs and are often HIV negative. Imaging findings can mimick cancer leading to delays in diagnosis and definitive treatment. Some experts have speculated that the spread of C. gattii is due to climate change, newly recognized genotypes that cause disease in temperate zones (genotype VGII), international travel, and improved awareness among physicians and veterinarians. We emphasize neurocritical and neurosurgical management, because patients with CNS involvement often have high intracranial pressures (ICP). Cryptococcus gattii patients often have elevated ICP without 'red flag' radiographic signs of elevated ICP such as ventriculomegaly, cerebral edema, or effaced basal cisterns. Therefore, diagnosis of high ICP should be suspected based on clinical symptoms such as incapacitating headaches, progressive visual loss and associated papilledema, and then confirmed by measuring the opening pressure with lumbar puncture (LP). Cerebral intraparenchymal deposition of the large cryptococcal polysaccharide capsule and cryptococcal organisms causes poor brain compliance leading to a 'frozen brain state.' Mortality rates and clinical outcomes are significantly improved with early diagnosis, antifungal therapies, steroids, and aggressive management of elevated ICP including cerebrospinal fluid (CSF) diversion by serial LP's, external ventricular drains and CSF shunts. Following institution of antifungal therapy, about 10% of patients can worsen due to immune reconstitution inflammatory syndrome which responds to steroids. We recommend neurocritical and neurosurgical management of C. gattii patients with CNS involvement and elevated ICP. There is often poor correlation between elevated ICP and neuroimaging due to the frozen brain state.
最初在热带地区报告的隐球菌感染现在在全球范围内诊断。方法：病例报告；文献回顾。尽管最初的报告描述了在澳大利亚和新几内亚等热带地区爆发的肺部和中枢神经系统（CNS）疾病，但现在很明显，隐球菌是一种全球性的、神经毒性病原体。与新型隐球菌不同，隐球菌患者更有可能在大脑和肺部出现 cryptococcomas，并且通常 HIV 阴性。影像学表现可能类似于癌症，导致诊断和明确治疗的延误。一些专家推测，隐球菌的传播是由于气候变化、在温带地区引起疾病的新识别基因型（基因型 VGII）、国际旅行以及医生和兽医意识的提高。我们强调神经危重症和神经外科管理，因为中枢神经系统受累的患者通常颅内压（ICP）较高。隐球菌患者通常 ICP 升高，而没有“警示”ICP 升高的放射学迹象，如脑室扩大、脑水肿或基底池消失。因此，应根据头痛、视力逐渐丧失和相关视乳头水肿等临床症状怀疑 ICP 升高，并通过腰椎穿刺（LP）测量开放压力来确认。大的隐球菌多糖胶囊和隐球菌生物体在脑实质内的沉积导致脑顺应性差，导致“脑冻结状态”。早期诊断、抗真菌治疗、类固醇和积极管理升高的 ICP，包括通过连续 LP、外部脑室引流和 CSF 分流来引流脑脊液，可显著改善死亡率和临床预后。在开始抗真菌治疗后，约 10%的患者可能会因免疫重建炎症综合征而恶化，该综合征对类固醇有反应。我们建议对中枢神经系统受累和 ICP 升高的隐球菌患者进行神经危重症和神经外科管理。由于脑冻结状态，ICP 升高与神经影像学之间通常相关性较差。
