Eimer Johannes, Vesterbacka Jan, Savitcheva Irina, Press Rayomand, Roshanisefat Homayoun, Nowak Piotr
Karolinska University Hospital Huddinge, Department of Infectious Diseases.
Institution for Medicine Huddinge, Unit of Infectious Diseases, Karolinska Institute, Huddinge, Sweden.
Medicine (Baltimore). 2018 Mar;97(12):e0162. doi: 10.1097/MD.0000000000010162.
Cognitive dysfunction is a common presenting symptom in patients with HIV/AIDS. It is usually directly associated with HIV infection or due to opportunistic infection. Rapidly progressive dementia, however, is rarely observed in acute HIV infection or during immune reconstitution. Recently, a case of Creutzfeld-Jakob disease (CJD) has been reported in a patient with chronic HIV infection. The incidence of CJD is not known to be increased among immunocompromised patients.
We here report the case of a 59-year-old male patient with a recent diagnosis of HIV/AIDS and Pneumocystis jiroveci pneumonia presenting with secondary behavioral changes and disorientation. Over the course of several weeks, progressive dementia developed characterized by apraxia, gait ataxia, and mutism.
After the exclusion of common HIV-associated neurologic conditions, the clinical course as well as findings on electroencephalogram (EEG), magnetic resonance imaging (MRI), and a positive 14-3-3 assay converged into a probable diagnosis of CJD. The diagnosis was later confirmed histopathologically.
Palliative care was provided, and the patient passed away within 2 months of symptom onset.
HIV/AIDS is an important stratifying condition during the work-up of many clinical syndromes including encephalopathy but may prematurely exclude important differential diagnoses. Non-opportunistic etiologies have to be considered as part of a secondary workup as this case of concomitant AIDS and CJD demonstrates. Rapidly progressive dementia should be distinguished from delirium as early as possible in order to be able to choose the correct diagnostic pathway. Despite the common occurrence of neurologic syndromes in the setting of immunodeficiency, an analytical diagnostic approach is advisable to minimize diagnostic bias.
认知功能障碍是HIV/AIDS患者常见的症状表现。它通常与HIV感染直接相关或由机会性感染引起。然而,快速进展性痴呆在急性HIV感染或免疫重建期间很少见。最近,有一例克雅氏病(CJD)在一名慢性HIV感染患者中被报道。免疫功能低下患者中CJD的发病率是否增加尚不清楚。
我们在此报告一例59岁男性患者,近期诊断为HIV/AIDS和耶氏肺孢子菌肺炎,伴有继发性行为改变和定向障碍。在数周内,逐渐发展为以失用症、步态共济失调和缄默症为特征的进行性痴呆。
在排除常见的HIV相关神经系统疾病后,临床病程以及脑电图(EEG)、磁共振成像(MRI)结果和14-3-3检测呈阳性,综合起来得出可能为CJD的诊断。该诊断后来经组织病理学证实。
提供了姑息治疗,患者在症状出现后2个月内去世。
HIV/AIDS是包括脑病在内的许多临床综合征检查过程中的重要分层因素,但可能过早排除重要的鉴别诊断。非机会性病因必须作为二次检查的一部分加以考虑,就像这例合并AIDS和CJD的病例所显示的那样。应尽早将快速进展性痴呆与谵妄区分开来,以便能够选择正确的诊断途径。尽管在免疫缺陷情况下神经综合征很常见,但建议采用分析性诊断方法以尽量减少诊断偏差。