Shen Guangxun, Dong Hanrong, Zhao Jingmin, Wu Si, Lee Kwee-Yum, Chi Lumei
Department of Neurology, China-Japan Union Hospital of Jilin University, Changchun, China.
Department of Orthopaedic Surgery and Musculoskeletal Medicine, University of Otago, Christchurch, New Zealand.
Heliyon. 2024 Aug 30;10(17):e37129. doi: 10.1016/j.heliyon.2024.e37129. eCollection 2024 Sep 15.
Delayed post-hypoxic leukoencephalopathy (DPHL) is characterized by a biphasic clinical course, with complete recovery from coma to a fully conscious state lasting one to four weeks (lucid interval), followed by abrupt neurological deterioration as an indirect consequence of hypoxic events like carbon monoxide poisoning and narcotic drug overdose. To our best knowledge, there are no documented cases in literature of choreoathetosis and dementia following poppy-induced DPHL with 14-3-3 protein in cerebrospinal fluid (CSF).
We report the case of a 70-year-old female who underwent cardiopulmonary resuscitation (CPR) due to overdose of homemade refined opium poppy paste two weeks prior to presentation. She presented a progressive cognitive decline, along with the development of apraxia and choreic movement affecting her tongue and bilateral upper and lower extremities. During the symptomatic phase, brain magnetic resonance imaging (MRI) showed bilateral symmetrical hyperintense signals mostly in central frontal, temporal, and parieto-occipital lobes in the diffusion weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) sequences which are the characteristic findings of DPHL. CSF routine analysis, as well as toxicology screening, autoimmune and paraneoplastic encephalitis panels were negative, but the presence of 14-3-3 protein in the CSF was detected. With steroid therapy, hyperbaric oxygen therapy and symptomatic treatment, she experienced gradual improvement in cognition, motivation, and psychomotor function.
DPHL represents a distinct form of encephalopathy characterized by unique clinical course and imaging features. It is the first report of DPHL with positive 14-3-3 protein in CSF. The potential of 14-3-3 protein as a biomarker for diagnosing DPHL and its ability to predict disease severity and prognosis warrants further research.
迟发性缺氧性白质脑病(DPHL)的临床病程呈双相性,患者从昏迷完全恢复至完全清醒状态,持续一至四周(清醒期),随后因一氧化碳中毒和麻醉药物过量等缺氧事件间接导致神经功能突然恶化。据我们所知,文献中尚无关于罂粟所致DPHL伴脑脊液(CSF)中14-3-3蛋白阳性后出现舞蹈手足徐动症和痴呆的病例报道。
我们报告一例70岁女性病例,该患者在就诊前两周因过量食用自制精制罂粟糊而接受了心肺复苏(CPR)。她出现了进行性认知衰退,同时出现失用症以及影响其舌头和双侧上下肢的舞蹈样动作。在症状期,脑部磁共振成像(MRI)在扩散加权成像(DWI)和液体衰减反转恢复(FLAIR)序列中显示双侧对称高信号,主要位于中央额叶、颞叶和顶枕叶,这是DPHL的特征性表现。脑脊液常规分析以及毒理学筛查、自身免疫性和副肿瘤性脑炎检测均为阴性,但脑脊液中检测到14-3-3蛋白存在。经过类固醇治疗、高压氧治疗和对症治疗,她的认知、动机和精神运动功能逐渐改善。
DPHL是一种独特的脑病形式,具有独特的临床病程和影像学特征。这是脑脊液中14-3-3蛋白阳性的DPHL的首例报告。14-3-3蛋白作为诊断DPHL的生物标志物的潜力及其预测疾病严重程度和预后的能力值得进一步研究。