Li Ying, Miao Yuanfeng, Yu Meng, Zhu Ying, Liang Zeyin, Wang Zhaoxia, Peng Qing
Department of Neurology, Peking University First Hospital, Beijing, China.
Department of Radiology, Peking University First Hospital, Beijing, China.
Front Neurol. 2021 Sep 17;12:743165. doi: 10.3389/fneur.2021.743165. eCollection 2021.
There are few reported cases of posterior reversible encephalopathy syndrome (PRES) combined with essential thrombocythemia (ET). We report a case of PRES-like syndrome in ET. A 60-year-old man with a history of hypertension and thrombocythemia presented with progressive visual loss after waking up; and neurological examination showed pupils were 3 mm and equally reactive to light, which suggested cortical blindness. Brain magnetic resonance imaging (MRI) revealed restricted diffusion in diffusion-weighted imaging (DWI) in the bilateral parietal and occipital lobes. Routine blood tests revealed a platelet count of 1,044 × 10/L. ET was diagnosed after exclusion of other causes. Electroencephalography (EEG) showed periodic triphasic waves in the occipital region. The lesions of the parietal and occipital lobes on MRI and periodic triphasic waves of EEG disappeared quickly, and patient's vision returned to normal after the treatment with hydroxyurea and sodium bicarbonate. The patient experienced hallucinatory palinopsia during the recovery of vision. ET may be a risk factor for PRES.
后部可逆性脑病综合征（PRES）合并原发性血小板增多症（ET）的报道病例较少。我们报告1例ET患者出现PRES样综合征。一名有高血压和血小板增多症病史的60岁男性，醒来后出现进行性视力丧失；神经检查显示瞳孔直径3mm，对光反应正常，提示皮质盲。脑磁共振成像（MRI）显示双侧顶叶和枕叶在扩散加权成像（DWI）上呈扩散受限。常规血液检查显示血小板计数为1044×10⁹/L。排除其他病因后诊断为ET。脑电图（EEG）显示枕区有周期性三相波。MRI上顶叶和枕叶的病变以及EEG的周期性三相波迅速消失，患者经羟基脲和碳酸氢钠治疗后视力恢复正常。患者在视力恢复过程中出现幻觉性视觉残留。ET可能是PRES的一个危险因素。
