Division of Rheumatology, Children's Hospital Los Angeles, CA, USA.
Clin Exp Rheumatol. 2012 Mar-Apr;30(2):272-6. Epub 2012 Apr 13.
We present the case of a 16-year-old patient with systemic lupus erythematosus who presented with altered mental status and regressive behaviour. She was worked up and empirically treated for common and opportunistic infectious agents. All work-up was negative and after an extensive course of antibiotics she was treated for neuropsychiatric lupus with cytoxan. She initially responded, but this was short-lived and she eventually became comatose and passed away. On brain biopsy she was found to have numerous trophozoites with round nucleus, prominent nucleolus and thin nuclear membrane. Methenamine silver stain showed encysted amoeba, corresponding with a diagnosis of acanthamoeba meningoencephalitis. Making the diagnosis of acanthamoeba meningoencephalitis requires a high degree of suspicion. Specific serum antibodies may not be a reliable measure in immunocompromised patients and trophozoites in CSF can be confused with monocytes. Brain biopsy may be required to make a definitive diagnosis. It is important for clinicians treating immunocompromised patients to keep this agent in mind in an immunocompromised patient with neurological manifestations. Acanthamoeba infections have only been reported in a small handful of patients and, to our knowledge, this is the first reported case in the United States.
我们报告了一例 16 岁系统性红斑狼疮患者，其表现为精神状态改变和退行性行为。对其进行了常规和机会性感染病原体的检查和经验性治疗。所有检查均为阴性，在使用广泛的抗生素治疗后，她被诊断为神经精神狼疮，并接受了环磷酰胺治疗。她最初有反应，但这种反应是短暂的，最终她陷入昏迷并死亡。在脑活检中，发现有许多滋养体，细胞核呈圆形，核仁明显，核膜薄。六亚甲基四胺银染色显示包囊阿米巴，诊断为棘阿米巴脑膜脑炎。诊断棘阿米巴脑膜脑炎需要高度怀疑。在免疫功能低下的患者中，特异性血清抗体可能不是可靠的衡量标准，CSF 中的滋养体可能与单核细胞混淆。可能需要进行脑活检以做出明确诊断。对于治疗免疫功能低下患者的临床医生来说，在免疫功能低下的有神经系统表现的患者中，应牢记这种病原体。棘阿米巴感染仅在少数患者中报告，据我们所知，这是美国首例报告病例。
