神经节苷脂联合大剂量免疫球蛋白治疗中重度新生儿缺氧缺血性脑病的临床研究

目的 探讨神经节苷脂联合大剂量免疫球蛋白治疗中重度新生儿缺氧缺血性脑病的临床效果.方法 随机抽取我院收治的80例中重度缺氧缺血性脑病新生儿,随机分为两组.对照组38例,行常规治疗;实验组42例,行神经节苷脂联合大剂量免疫球蛋白治疗.随访1～2个月,比较两组患儿临床神经症状恢复时间和新生儿行为神经评分.结果 两组患儿神经症状恢复时间和治疗后NBNA评分均有显著差异(P＜0.05),且实验组疗效优于对照组.结论 应用神经节苷脂联合大剂量免疫球蛋白治疗新生儿缺血缺氧性脑病可有效缩短病程,治疗效果好.     

皮层下动脉硬化性脑病的临床CT特征

目的:分析117例SAE的临床表现及CT特征。方法:本组117病例中,男81例,女36例,年龄范围56～81岁,观察临床CT特点进行分析。结果:其临床表现为智力减退、神经精神障碍、下肢无力及行走障碍、偏瘫。CT表现为侧脑室周围分布多发斑片状或弥漫性、相互融合低密度阴影,边界不清,多为双侧对称分布,以两侧脑室额角周围最易受累且最早出现,其次为放射冠,侧脑室三角区及枕角周围、半卵圆中心,多伴有脑萎缩及脑梗塞。结论:CT检查能够准确诊断SAE的病变程度,高血压和脑动脉硬化患者早期CT检查,防治SAE有重要临床价值。     

Isolated angiitis of the CNS and bacterial endocarditis: similarities and differences

Both isolated angiitis of the central nervous system (IAN) and bacterial endocarditis (BE) may present with similar clinical and auxiliary findings. The differentiation is extremely important because of the different treatment regimens. We compared the findings of six patients with biopsy-proven IAN with the data of six patients with BE. Patients with IAN were younger (27–62 years) and presented with multiple strokes (n = 4), intracerebral hemorrhage (n = 1), epileptic seizures (n = 2), or encephalopathy (n = 1). All IAN patients had pathologic cerebrospinal fluid (CSF) findings (pleocytosis n = 5; protein elevation n = 4), and angiography revealed multilocular stenoses in two cases while digital subtraction angiography was normal in four. BE patients (32–77 years) presented multiple (n = 3) or single ischemic strokes (n = 2) or encephalopathy and headache (n = 2). While all patients showed inflammatory serum findings (C-reactive protein n = 6, leucocytosis n = 4), CSF-pleocytosis was present in two cases only. Angiography revealed a vasculitic pattern in two patients. The diagnosis of BE was etablished based on transesophageal echocardiography and blood cultures. Leptomeningeal and brain biopsies performed in two cases were normal. Both IAN and BE may present multiple strokes and encephalopathy. The frequency of a vasculitic pattern in angiography is similar in both conditions. While inflammatory serum findings are the rule in BE, pathologic CSF findings were present in all IAN patients. Transesophageal echocardiography and blood cultures should be performed in order to diagnose or exclude BE. Without brain biopsy, immunosuppressive therapy may be dangerous in suspected IAN.     

Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome

An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. Blood chemistry revealed hyperammonemia of 2420 μg/l as a cause of this altered mental status disturbance. Superior mesenteric arteriography revealed large portal-systemic shunts in venous phase as a cause of hyperammonemia. To control blood ammonia level, we placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device. This case illustrates that portal-systemic shunt may result in hyperammonemia leading to altered mental status long after a Fontan-type operation.     

Co-administration of C-Phycocyanin ameliorates thioacetamide-induced hepatic encephalopathy in Wistar rats

Fulminant hepatic failure (FHF) is a condition with a sudden onset of necrosis followed by degeneration of hepatocytes, without any previously established liver disease, generally occurring within hours or days. FHF is associated with a wide spectrum of neuropsychiatric alterations ranging from stupor to coma, culminating in death. In the present study FHF was induced in rats by the administration of thioacetamide (TAA). Oxidative stress is thought to play a prominent role in the pathophysiology of cerebral changes during FHF leading to the assumption that antioxidants might offer protection. Hence, in the present study the protective effect of C-Phycocyanin (C-PC), a natural antioxidant, was evaluated on TAA-induced tissue damage. C-Phycocyanin was administered intraperitoneally twice at 24 h interval (50 mg/kg body weight) along with the hepatotoxin TAA (300 mg/kg body weight). The animals were sacrificed 18 h after the second injection of TAA treatment and various biochemical parameters were analysed in liver, serum and brain tissues. These studies revealed significant prevention of TAA-induced liver damage by C-PC, as evidenced by a) increase in survival rate; b) the prevention of leakage of liver enzymes (AAT and AST) and ammonia into serum; c) increase in prothrombin time and d) liver histopathology. Ultrastructural studies of astrocytes of different regions of brain clearly showed a decrease in edema after C-PC treatment. TAA-induced histopathological lesions in different regions of the brain namely cerebral cortex, cerebellum and pons medulla were significantly reduced by the co-administration of C-PC with TAA. Further C-PC treatment resulted in a) decrease in the levels of tryptophan and markers of lipid peroxidation and b) elevation in the activity levels of catalase, glutathione peroxidase in different regions of brain. These studies reveal the potential of C-PC in ameliorating TAA-induced hepatic encephalopathy by improving antioxidant defenses.     

Neuronal cell death in hepatic encephalopathy

It is generally assumed that neuronal cell death is minimal in liver failure and is insufficient to account for the neuropsychiatric symptoms characteristic of hepatic encephalopathy. However, contrary to this assumption, neuronal cell damage and death are well documented in liver failure patients, taking the form of several distinct clinical entities namely acquired (non-Wilsonian) hepatocerebral degeneration, cirrhosis-related Parkinsonism, post-shunt myelopathy and cerebellar degeneration. In addition, there is evidence to suggest that liver failure contributes to the severity of neuronal loss in Wernicke’s encephalopathy. The long-standing nature of the thalamic and cerebellar lesions, over 80% of which are missed by routine clinical evaluation, together with the probability that they are nutritional in origin, underscores the need for careful nutritional management (adequate dietary protein, Vitamin B₁) in liver failure patients. Mechanisms identified with the potential to cause neuronal cell death in liver failure include NMDA receptor-mediated excitotoxicity, lactic acidosis, oxidative/nitrosative stress and the presence of pro-inflammatory cytokines. The extent of neuronal damage in liver failure may be attenuated by compensatory mechanisms that include down-regulation of NMDA receptors, hypothermia and the presence of neuroprotective steroids such as allopregnanolone. These findings suggest that some of the purported “sequelae” of liver transplantation (gait ataxia, memory loss, confusion) could reflect preexisting neuropathology.     

Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES)

Purposes:   To describe the clinical spectrum and to evaluate the efficacy of different therapeutic agents in children with electrical status epilepticus in sleep (ESES).
Methods:   Clinical data of all patients with ESES (not including patients with Landau-Kleffner syndrome) in four pediatric neurology outpatient clinics were analyzed. Thirty patients with ESES had been treated between 1994 and 2007.
Results:   Eleven (37%) children had benign partial epilepsies of childhood, five (17%) had cerebral palsy, five (17%) had hydrocephalus, one (3%) had schizencephaly, one (3%) had prenatal parenchymal bleeding, and the etiology was unclear in seven (23%). The duration of ESES ranged between 2 and 60 months. The antiepileptic drugs that were found to be efficacious were: levetiracetam (41%), clobazam (31%), and sulthiame (17%). Valproic acid, lamotrigine, topiramate, and ethosuximide showed no efficacy. Steroids were efficacious in 65%; immunoglobulins were efficacious in 33%. High-dose diazepam was efficacious in 37%, but all the children had temporary response. Seventeen patients (57%) had cognitive deterioration, whereas the rest presented with regression in attention, speech, communication, and behavior. Fourteen children had permanent cognitive deficit. There was a significant correlation (p = 0.029) between the duration of ESES and residual intellectual deficit at follow-up.
Conclusions:   ESES reflects an evolution of benign partial epilepsy of childhood in more than one-third of the patients, whereas there is an underlying structural brain anomaly in another one-third. The most efficacious antiepileptic drugs (AEDs) are levetiracetam and clobazam. The duration of ESES correlated significantly with residual intellectual deficit at follow-up.     

碘海醇致1例严重神经系统不良反应并文献复习

目的：提高对碘海醇神经系统严重不良反应的认识。方法：报告1例超剂量使用碘海醇行腰椎脊髓造影术致患者出现脑病病例,并进行文献复习。结果：碘海醇具有神经系统毒性,过量使用加大出现严重不良反应的风险。结论：使用碘海醇之前,告知患者及家属风险,了解患者过敏史,签署知情同意书,做好急救准备,有助于减少可能出现的不良反应和医患纠纷。