He Yun-Sen, Qin Xiao-Hong, Feng Min, Huang Qin-Jiang, Zhang Meng-Jun, Guo Li-Li, Bao Ming-Bin, Tao Ye, Dai Hong-Yuan, Wu Bo
Department of Neurosurgery, Sichuan Lansheng Brain Hospital & Shanghai Lansheng Brain Hospital Investment Co., Ltd., Chengdu 610036, Sichuan Province, China.
Department of Psychiatry, Sichuan Provincial Center for Mental Health, Chengdu 610072, Sichuan Province, China.
World J Clin Cases. 2024 Apr 26;12(12):2065-2073. doi: 10.12998/wjcc.v12.i12.2065.
Human immunodeficiency virus (HIV)-associated dementia (HAD) is a subcortical form of dementia characterized by memory deficits and psychomotor slowing. However, HAD often presents with symptoms similar to those of Creutzfeldt-Jakob disease (CJD), particularly in patients with acquired immune deficiency syndrome (AIDS).
We report the case of a 54-year-old male who exhibited cognitive dysfunction and secondary behavioral changes following HIV infection and suspected prion exposure. The patient was diagnosed with HIV during hospitalization and his cerebrospinal fluid tested positive for 14-3-3 proteins. His electroencephalogram showed a borderline-abnormal periodic triphasic wave pattern. Contrast-enhanced magnetic resonance imaging revealed moderate encephalatrophy and demyelination. Initially, symptomatic treatment and administration of amantadine were pursued for presumed CJD, but the patient's condition continued to deteriorate. By contrast, the patient's condition improved following anti-HIV therapy. This individual is also the only patient with this prognosis to have survived over 4 years. Thus, the diagnosis was revised to HAD.
In the diagnostic process of rapidly progressive dementia, it is crucial to rule out as many potential causes as possible and to consider an autopsy to diminish diagnostic uncertainty. The 14-3-3 protein should not be regarded as the definitive marker for CJD. Comprehensive laboratory screening for infectious diseases is essential to enhance diagnostic precision, especially in AIDS patients with potential CJD. Ultimately, a trial of diagnostic treatment may be considered when additional testing is not feasible.
人类免疫缺陷病毒(HIV)相关痴呆(HAD)是一种皮质下痴呆形式,其特征为记忆缺陷和精神运动迟缓。然而,HAD常表现出与克雅氏病(CJD)相似的症状,尤其是在获得性免疫缺陷综合征(AIDS)患者中。
我们报告了一例54岁男性病例,该患者在感染HIV并疑似接触朊病毒后出现认知功能障碍和继发性行为改变。患者住院期间被诊断为HIV感染,其脑脊液14-3-3蛋白检测呈阳性。脑电图显示临界异常的周期性三相波型。对比增强磁共振成像显示中度脑萎缩和脱髓鞘。最初,针对疑似CJD进行了对症治疗并给予金刚烷胺,但患者病情持续恶化。相比之下,抗HIV治疗后患者病情有所改善。该患者也是唯一一名存活超过4年的具有这种预后的患者。因此,诊断修订为HAD。
在快速进展性痴呆的诊断过程中,尽可能排除多种潜在病因并考虑进行尸检以减少诊断不确定性至关重要。14-3-3蛋白不应被视为CJD的确诊标志物。全面的传染病实验室筛查对于提高诊断准确性至关重要,尤其是在可能患有CJD的AIDS患者中。最终,在无法进行额外检测时可考虑进行诊断性治疗试验。