Severe psychomotor developmental delay resulting from early postnatal (within 3 months) seizures can be diagnosed as Early-Infantile Developmental and Epileptic encephalopathies (EIDEE). Its primary etiologies include structural, hereditary, metabolic and etc. The main pathogenesis may be related to the inhibition of normal physiological activity of the brain by abnormal electrical activity and the damage of the brain neural network. Ohtahara syndrome and Early Myoclonic Encephalopathy (EME) are typical types of EIDEE. The principle of treatment is to improve the cognitive and developmental function by controlling frequent seizures. When the seizure is difficult to control with drugs, surgical evaluation should be performed as soon as possible, and surgical treatment is the first choice for patients suitable for surgery. The types of surgery can be divided into excision surgery, dissociation surgery, neuromodulation surgery and etc. The current status of surgical treatment of EIDEE was described, and the curative effect of surgical treatment was explored, so as to help clinicians choose appropriate treatment methods.
【摘要】 目的 探讨脑后部可逆性脑病综合征（posterior reversible encephalopathy syndrome，PRES）的临床及MRI表现，提高对PRES的认识。 方法 回顾性分析2007年1月-2010年12月期间5例PRES患者的MRI资料并进行追踪随访。5例PRES患者中男1例，女4例，肾性高血压及产后子痫各1例、妊娠高血压3例。5例均行MRI多序列平扫检查及弥散加权成像（diffusion weighted imaging，DWI），其中4例同时行磁共振血管造影（magnetic resonance angiography，MRA），2例行MRI增强检查。 结果 5例发病时均有高血压，以突发头痛、子痫或癫痫发作、意识障碍及视觉障碍为临床特点，及时正确治疗后症状于3~5 d左右消失，1例遗留肢体功能障碍。MRI显示5例多发病灶主要位于双侧顶枕叶皮质下白质内，额叶及颞叶后部各2例，两侧小脑及脑干1例，皮质受累2例，病变呈长T1、长T2信号、液体衰减反转恢复序列呈高信号，5例患者病灶DWI图呈高或等信号，表观弥散系数（apparent diffusion coefficient，ADC）图呈略高信号；1例顶叶及1例基底节区部分病灶DWI图呈高信号，ADC图呈低信号，提示弥散受限。2例增强无强化，脑膜呈线状强化。随访MRI显示3例病灶完全消失。1例左侧基底节区部分病灶发展为脑梗死。 结论 PRES是一种临床-影像综合征，正确认识这一综合征对其早期诊断和治疗具有非常重要的意义。MRI能够提供较为可靠明确的诊断，其扩散成像对判断PRES预后亦很有价值。【Abstract】 Objective To investigate the clinical features and MRI manifestations of patients with posterior reversible encephalopathy syndrome (PRES), in order to improve its recognition. Methods From January 2007 to December 2010, MRI data of 5 patients with PRES were retrospectively analyzed and the patients were followed up. Among them, one was male and four were female. There were one case of postpartum eclampsia, one of renal hypertention, and 3 of pregnancy-induced hypertension. All the five patients were examined by MRI diffusion weighted imaging (DWI), among whom four underwent additional magnetic resonance angiography (MRA) and one underwent enhanced MRI. Results All the five patients with PRES had hypertension and their clinical symptoms were characterized by sudden occurrence of headache, eclampsia or seizure of epilepsy, altered mental status, and visual disturbances. Clinical symptoms vanished about 3-5 days after prompt and appropriate treatment except one case of residual body function failure. MRI scanning demonstrated multifocal subcortical white lesions in bilateral parieto-occipital lobes in five patients, in bilateral frontal lobes in two patients, in bilateral post temporal lobes in two patients, in bilateral cerebellum in one patient, and in bilateral brainstem in one patient, and cortical involvement occurred in one patient. Lesions appeared as hyperintense signals on fluid attenuated inversion recovery (FLAIR) and T2-weighted images, hypointense signals on T1-weighted images, normal or increased intensity on DWI, and mild hypointensity on apparent diffusion coefficient (ADC) maps. Some lesions in parietal lobes or basal ganglia showed hyperintensity on DWI and isointensity on ADC maps. The lesions in two patients appeared unenhanced with gadolinium enhancement and the meninges assumed the linear strengthening. Follow-up MRI showed that foci in three patients vanished completely, and the focus in the left basal ganglia in one patient developed into cerebral infarction. Conclusions PRES is a clinical-neuroradiological transient condition. Correctly understanding this syndrome is meaningful for its early diagnosis. MRI can provide a more reliable diagnosis, and its proliferation image formation is also valuable in predicting the PERS prognosis.
Objective To evaluate the efficacy and safety of Shengmai injection for hypoxic-ischemic encephalopathy (HIE). Methods We searched MEDLINE (1966 to February 2007), EMBASE (1980 to February 2007), CBM (1978 to 2006), CNKI (1979 to February 2007), VIP (1989 to February 2007), and handsearched five Journals on Pediatrics. We evaluated features of quality of included studies, including randomization, blinding, allocation concealment and loss of follow-up. Meta-analyses were performed using The Cochrane Collaboration’s RevMan 4.2.8. Results Seven randomized controlled trials were included. The cure rate on day 5 in the Shengmai injection group was higher than in the control group (RR 1.55, 95%CI 1.25 to 1.93), but this rate was similar on day 10 (RR 0.74, 95%CI 0.43 to 1.29). No significant difference in cure rate was noted between the Shengmai injection group and naloxone group (RR 0.88, 95%CI 0.53 to 1.46). No significant differences were observed in mortality (RR0.44, 95%CI 0.16 to 1.19) and mutilation rate (RR 0.58, 95%CI 0.21 to 1.56) between the Shengmai injection group and the control group. For those babies suffering from HIE combined with myocardial damage, Shengmai injection could speed up the recovery of ECG (WMD=–2.02, 95%CI –2.76 to –1.28) and myocardial enzymogram (CK-MB: WMD= –4.78, 95%CI –6.77 to –2.79; CK-BB: WMD=–2.68, 95%CI –4.58 to –0.78). Significant differences in NBNA score were noted between the Shengmai injection group and the control group on day 5 (WMD=4.05, 95%CI 2.47 to 5.63) and day 10 (WMD=3.50, 95%CI 2.26 to 4.74). No fatal side effects were reported. Conclusions Shengmai injection has certain therapeutic values in treating HIE. Shengmai injection can speed up the recovery ECG, CK-BM and CK-BB of HIE patients, especially in those who have myocardial damage. Shengmai injection can also improve the NBNA score. However, because of the low statistical power and high risks for selection bias, performance bias and measurement bias in the included trials, these conclusions need to be interpreted cautiously.
Objective To observe the serumlevel of neuron-specific enolase( NSE) in patients with pulmonary encephalopathy and its changes after treatment with mechanical ventilation. Methods Twentyone patients with pulmonary encephalopathy were enrolled. Glasgow coma scale( GCS) , serumNSE level, and arterial blood gas were evaluated at three time-points: before mechanical ventilation, after 12 hours mechanical ventilation, and the moment of consciousness. Results 18 patients recovered consciousness, and 3 patients remained in persistent coma and died. GCS and arterial blood gas improved obviously after 12 hours mechanical ventilation. Meanwhile, the serumNSE concentration decreased significantly after 12 hours mechanical ventilation [ ( 24. 54 ±6. 65) μg/L] and at the moment of consciousness [ ( 14. 19 ±2. 91) μg/L] compared with before mechanical ventilation( P lt; 0. 05, P lt; 0. 01) . Conclusion Dynamic measurment of serumNSE may be a useful biomarker for assessing the severity of cerebral injury and predicting prognosis.