【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.
Seventeen cases of pancreatic encephalopathy (PE) with acute pancreatitis were studied retrospectively. It was found that on the basis of brain damage caused by pancreatic enzyme, many factor might play a role in the development of PE. It suggests that PE should not be accepted as an operative indication separately in severe acute pancreatitis. Chinese medicine can benefit the patient in the treatment of this disease. Operation is the only choice while patient get worsened even after appropriate and enough nonoperative therapy, as well as while pancreatic necrosis become infected or pancreatic abcess formed. Mortality of PE in this series is 52.9%, slightly less than the level (66.7%-100%) reported by other authors.
Objective To evaluate the efficacy and safety of L-ornithine-L-aspartate (LOLA) in the treatment of hepatic encephalopathy (HE). Methods Such databases as PubMed, EMbase, Web of Science, CENTRAL, Chinese Journals Full-text Database, CBM and WanFang Data were searched from the date of their establishment to November 30, 2011 to collect the randomized controlled trials (RCTs) on LOLA in treating HE. The quality of included studies was evaluated by two reviewers independently, data were extracted and cross-checked, and then meta-analysis was performed using Review Manager 5.0 software. Results Among the total six included RCTs, 432 patients were diagnosed as type-C HE and 185 were diagnosed as type-A HE. Compared with the placebo group, for the patients with type-C HE, LOLA could significantly reduce the serum ammonia level (WMD=16.60, 95%CI 8.34 to 24.85, Plt;0.000 1) and the time of number connection test-A (NCT-A) (WMD=9.6, 95%CI 5.26 to 13.93, Plt;0.00 01), and it could also effectively improve the clinical remission rate (RR=1.36, 95%CI 1.06 to 1.73, P=0.01). However, no significant differences were found between the two groups for the patients with type-A HE (Pgt;0.05). Conclusion LOLA is effective for the patients with type-C hepatic encephalopathy, and it could be regarded as a choice in clinic. However, more high-quality RCTs are needed to further evaluate the effect of LOLA in treating type-A hepatic encephalopathy.
Objective To explore the efficacy and safety of the ketogenic diet (KD) in the treatment of genetic developmental and epileptic encephalopathy (DEE). Methods Clinical data from 42 children with genetically confirmed refractory epileptic encephalopathy treated in the Department of Neurology, Jinan Children’s Hospital, between January 2021 and October 2023 were retrospectively analyzed. A classic KD protocol was implemented, and outcomes including seizure frequency, electroencephalogram (EEG) improvement, and adverse reactions were observed at 3, 6, and 12 months post-treatment. Results Among the 42 children, the seizure-free rates at 3, 6, and 12 months of KD treatment were 16.7%, 16.7%, and 14.3%, respectively, while the effective seizure control rates were 69.0%, 52.4%, and 35.7%. At 3 months, comparison of baseline characteristics between the effective and ineffective groups showed no statistically significant differences in gender (P=0.095), age at onset (P=0.648), age at KD initiation(P=0.768), disease duration before KD (P=0.519), presence of abnormal brain MRI findings (P=0.226), epilepsy syndrome classification(P=0.344), or ion channel gene involvement (P=0.066). EEG improvement rates at 6 and 12 months were 54.2% (24 cases) and 42.8% (14 cases), respectively. Retention rates for KD at 3, 6, and 12 months were 100.0%, 71.4%, and 42.8%. Adverse reactions occurred in 7 patients (16.7%), primarily gastrointestinal symptoms (vomiting, constipation, diarrhea; 6 cases) and elevated uric acid (1 case), with no severe adverse events reported. Conclusion KD is an effective treatment for genetic DEE with favorable short-term safety, though long-term adherence requires attention.
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.
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.
Developmental epileptic encephalopathies (DEEs) are a group of disorders characterized by early-onset seizures, abnormal electroencephalogram (EEG) patterns, and developmental delay or regression. They are characterized by complex etiology and are often refractory to treatment, severely impacting affected children, particularly infants and toddlers, and pose a challenge in pediatric neurology. In recent years, with the rise of precision medicine, an increasing number of pathogenic genes associated with DEEs have been discovered. However, the specific pathogenic mechanisms and signaling pathways of these genes in the body still require further investigation. This article primarily discusses the genetic patterns of DEEs and the selection of genetic testing, emphasizing the timing of genetic testing assisted by the epilepsy phenotype, especially in DEEs associated with single-gene mutations and new therapeutic drugs, to aid in clinical decision-making for DEEs. It also introduces the use of neurobiological models for DEE research to effectively advance epilepsy research, thereby enabling targeted gene therapy.
Objective To construct a nomogram model for predicting delayed encephalopathy after acute carbon monoxide poisoning (DEACMP) in emergency departments. Methods All patients with acute carbon monoxide poisoning who visited the Department of Emergency of Zigong Fourth People’s Hospital between June 1st, 2011 and May 31st, 2023 were retrospectively enrolled and randomly divided into a training set and a testing set in a 6∶4 ratio. LASSO regression was used to screen variables in the training set to establish a nomogram model for predicting DEACMP. The discrimination, calibration, and clinical practicality were compared between the nomogram and Glasgow Coma Scale (GCS) in the training and testing sets. Results A total of 475 patients with acute carbon monoxide poisoning were included, of whom 41 patients had DEACMP. Age, GCS and aspartate aminotransferase were selected as risk factors through LASSO regression, and a nomogram model was constructed based on these factors. The areas under the receiver operating characteristic curves for nomogram and GCS to predict DEACMP in the training set were 0.897 [95% confidence interval (CI) (0.829, 0.966)] and 0.877 [95%CI (0.797, 0.957)], respectively; and those for nomogram and GCS to predict DEACMP in the testing set were 0.925 [95%CI (0.865, 0.985)] and 0.858 [95%CI (0.752, 0.965)], respectively. Compared with GCS, the performance of nomogram in the training set (net reclassification index=0.495, P=0.014; integrated discrimination improvement=0.070, P=0.011) and testing set (net reclassification index=0.721, P=0.004; integrated discrimination improvement=0.138, P=0.009) were both positively improved. The calibration of nomogram in the training set and testing set was higher than that of GCS. The decision curves in the training set and testing set showed that the nomogram had better clinical net benefits than GCS. Conclusion The age, GCS and aspartate aminotransferase are risk factors for DEACMP, and the nomogram model established based on these factors has better discrimination, calibration, and clinical practicality compared to GCS.
目的 探讨5例特重型胰腺炎的特点及治疗方法。方法 我院2001年8月至2003年8月共收治特重型胰腺炎患者5例。其中入院后18 h内心跳、呼吸骤停3次的重症急性胰腺炎（SAP）1例，治疗以及时血液滤过和心、肺、脑复苏为重点； SAP并发胰性脑病2例，以大剂量维生素B1的补充，或足量补给浓缩红细胞为治疗重点； 并发多个器官功能障碍的暴发性胰腺炎（FAP）2例，治疗重点是血液滤过和防治多器官功能衰竭的级联放大反应，其中1例以高渗性糖昏迷为主要表现，治疗重点是内稳态的纠正，血液滤过，重要器官功能维护。结果 5例特重型胰腺炎患者均治愈，平均住院时间为32.2 d。结论 器官功能的复苏和维护、外科ICU监护、短时血液滤过、内稳态的纠正、中西药综合治疗及病因、对症的个体化治疗是特重型胰腺炎的重要治疗措施。