Khan Raja B, Sadighi Zsila S, Zabrowski Jennifer, Gajjar Amar, Jeha Sima
Division of Neurology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Department of Pediatric Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee.
Pediatr Blood Cancer. 2016 Mar;63(3):523-6. doi: 10.1002/pbc.25790. Epub 2015 Oct 15.
Diagnosis of posterior reversible encephalopathy syndrome (PRES) requires presence of headache, seizures, impaired vision, or altered mentation accompanied by specific imaging findings. We aimed to study long-term clinical and radiologic outcome of PRES in children with cancer to augment limited available data.
Retrospective review of children with cancer who were diagnosed with PRES.
We identified PRES in 21 males and 16 females among 5,217 children treated during the study period. Median time from cancer diagnosis to PRES was 6.6 months in 25 leukemia (1.6%), five brain tumor (0.3%), and seven other solid tumor (0.4%) patients; P = <0.0001 for leukemia versus all other tumors. Symptoms included seizures (97%), headaches (40%), altered mentation (68%), and vision impairment (27%). Hypertension was seen in 97%, and steroids use was seen in 78%. Headaches, visual disturbance, and mental status resolved within a median of <3 days, whereas epilepsy developed in 19%. T2 hyperintense signal was present in 100% of occipital, 47% of temporal, 75% of parietal, and 55% of frontal lobes, as well as 22% of cerebellum and 5% of basal ganglia. Follow-up magnetic resonance imaging (MRI) in 34 patients showed partial or complete T2 resolution in 79%, development of laminar necrosis in five, microhemorrhages in six, and focal atrophy in three.
PRES in children is more common in hematological malignancy compared with other tumors and is associated with hypertension and steroid use. Seizure is the most common acute manifestation. Most MRI changes resolve, but persistent imaging abnormality and epilepsy may develop in a significant minority.
后部可逆性脑病综合征(PRES)的诊断需要存在头痛、癫痫发作、视力障碍或精神状态改变,并伴有特定的影像学表现。我们旨在研究癌症患儿PRES的长期临床和影像学结局,以补充有限的现有数据。
对诊断为PRES的癌症患儿进行回顾性研究。
在研究期间接受治疗的5217名儿童中,我们确定了21名男性和16名女性患有PRES。25例白血病(1.6%)、5例脑肿瘤(0.3%)和7例其他实体瘤(0.4%)患者从癌症诊断到PRES的中位时间为6.6个月;白血病与所有其他肿瘤相比,P = <0.0001。症状包括癫痫发作(97%)、头痛(40%)、精神状态改变(68%)和视力障碍(27%)。97%的患者出现高血压,78%的患者使用类固醇。头痛、视觉障碍和精神状态在中位时间<3天内缓解,而19%的患者发生癫痫。100%的枕叶、47%的颞叶、75%的顶叶和55%的额叶以及22%的小脑和5%的基底神经节出现T2高信号。34例患者的随访磁共振成像(MRI)显示79%的患者T2部分或完全消退,5例出现层状坏死,6例出现微出血,3例出现局灶性萎缩。
与其他肿瘤相比,PRES在儿童血液系统恶性肿瘤中更常见,且与高血压和类固醇使用有关。癫痫发作是最常见的急性表现。大多数MRI改变会消退,但少数患者可能会出现持续的影像学异常和癫痫。