Bauer Angela, Loos Sebastian, Wehrmann Carola, Horstmann Dirk, Donnerstag Frank, Lemke Johanna, Hillebrand Georg, Löbel Ulrike, Pape Lars, Haffner Dieter, Bindt Carola, Ahlenstiel Thurid, Melk Anette, Lehnhardt Anja, Kemper Markus J, Oh Jun, Hartmann Hans
Department of Pediatrics, University Medical Center Hamburg, Hamburg, Germany.
Pediatr Nephrol. 2014 Sep;29(9):1607-15. doi: 10.1007/s00467-014-2803-x. Epub 2014 Mar 25.
The aim of this study was to analyze the neurological involvement and outcome in pediatric patients with hemolytic uremic syndrome (HUS) during the 2011 epidemic caused by Escherichia coli O104:H4.
Clinical data and data from magnetic resonance imaging (MRI) scans and electroencephalography (EEG) during the acute phase of the disease and during follow-up at 3 and 6 months were analyzed in 50 patients. Twenty-five of these patients underwent neuropsychological testing (WISC IV) during follow-up.
Neurological involvement (stupor or coma, seizures, visual disturbances, paresis, myocloni) was initially observed in 14/50 (28%) patients. One patient died. EEG abnormalities were more frequent in patients with neurological involvement than in those without (12/14 vs. 13/25, respectively). Cranial MRI scans were analyzed in nine patients with neurological involvement, of whom five showed abnormal findings. At the 3- and 6-month follow-ups, EEG abnormalities were found in 14/40 (35%) and 7/36 (19%) patients, respectively, whereas 28/42 (67%) and 17/39 (44%) patients, respectively, complained about on-going reduced performance. Neuropsychological testing showed a slightly lower global intelligence quotient in patients with neurological involvement versus those without (113.4 ± 2.8 vs. 119.4 ± 1.8, respectively).
Neurological involvement was frequent in our cohort. Accordingly, the incidence of pathological EEG findings was high, even in patients without clinical signs of neurological involvement. Nevertheless, major neurological sequelae were rare, and neuropsychological outcome was favorable after 6 months.
本研究旨在分析2011年由大肠杆菌O104:H4引起的疫情期间溶血尿毒综合征（HUS）儿科患者的神经受累情况及预后。
分析了50例患者在疾病急性期以及3个月和6个月随访期间的临床数据、磁共振成像（MRI）扫描数据和脑电图（EEG）数据。其中25例患者在随访期间接受了神经心理学测试（韦氏儿童智力量表第四版）。
最初在14/50（28%）的患者中观察到神经受累（昏迷或昏睡、癫痫发作、视觉障碍、轻瘫、肌阵挛）。1例患者死亡。神经受累患者的脑电图异常比未受累患者更常见（分别为12/14和13/25）。对9例神经受累患者进行了头颅MRI扫描，其中5例有异常发现。在3个月和6个月的随访中，分别有14/40（35%）和7/36（19%）的患者发现脑电图异常，而分别有28/42（67%）和17/39（44%）的患者抱怨持续存在功能下降。神经心理学测试显示，神经受累患者的总体智商略低于未受累患者（分别为113.4±2.8和119.4±1.8）。
在我们的队列中神经受累很常见。因此，即使在没有神经受累临床体征的患者中，病理性脑电图结果的发生率也很高。然而，严重的神经后遗症很少见，6个月后神经心理学预后良好。
