急性一氧化碳中毒迟发脑病30例高压氧治疗效果的临床分析

目的：探讨急性一氧化碳中毒迟发脑病的发病机制,临床表现,影像学改变,高压氧治疗和预后,加深对这种疾病的认识。方法对30例急性一氧化碳中毒迟发脑病患者的临床分析。结果急性一氧化碳中毒迟发脑病患者临床表现为情感障碍,肌张力增高,震颤及步态不稳等表现,头颅CT及MRI常显示基底节区,放射冠低密度改变,脑电图均有不同程度慢波表现。经高压氧治疗痊愈10例,好转12例,有所好转8例。结论急性一氧化碳中毒迟发脑病是急性一氧化碳中毒较为常见的一种并发症,治疗上应遵循早,足,综合治疗原则,尤其早期高压氧的综合治疗对预防和治疗一氧化碳中毒迟发脑病有临床意义。     

橄榄桥脑小脑萎缩的临床及影像学分析

目的：分析橄榄桥脑小脑萎缩（OPCA）病人早期的临床表现及MRI特征,以利早期诊断。方法：对20例OPCA病人的临床表现及影像学特征进行回顾性分析。结果：OPCA病人男性多于女性,平均年龄56．5岁,其临床表现多种多样,以小脑症状、植物神经症状及锥体外系症状多见,头颅MRI以小脑和脑干萎缩为主,大脑皮质萎缩轻;其中11例病人T2WI上可见位于桥脑至延髓上部贯穿于脑干前后径的连续性细线样高信号,3例病人可见脑桥T2WI显示“十字”征。结论：成年人出现小脑性共济失调、植物神经功能紊乱和锥体外系症状,应高度怀疑OPCA,MRI有助于OPCA的诊断;“纵线”征和“十字”征在OPCA的诊断和鉴别诊断中意义重大。     

急性病毒性脑炎并发癫(癎)症状患儿的脑电图分析

目的：探讨小儿急性病毒性脑炎并发癫癎的临床表现及与脑电图（EEG）的关系。方法：对38例出现并发癫癎的急性病毒性脑炎患儿EEG、头颅MRI及CT的表现进行分析。结果：在出现癫癎症状的急性病毒性脑炎患儿的EEG描记中，表现为低至中波幅慢波增多，呈弥漫性或局限性异常者占87％，出现癫癎样放电者占13%，头颅MRI及CT检出继发性癫癎的病灶者分别占80％及50％。结论：在小儿急性病毒性脑炎并发癫癎症状时，EEG是一项重要的临床检查项目，对了解脑功能状况有重要的参考作用。     

76例颞叶癫癎患者的临床表现与EEG、MRI改变

目的：分析颞叶癫癎患者的临床与脑电图（EEG）和MRI表现。方法：对76例颞叶癫癎患者的各种临床与EEG和MRI资料进行回顾性分析。结果：颞叶癫癎在临床上以复杂部分性发作为代表,部分患者具有先兆症状,其后出现自动症、感觉性症状、精神情感异常、自主神经症状及肢体抽搐等临床表现,其蝶骨电极EEG可记录到癎性异常波,头颅MRI多可见颞叶萎缩和海马硬化。结论：颞叶癫癎是常见的一种癫癎类型,其临床表现复杂多样,蝶骨电极EEG及头颅MRI对反映患者的脑电活动及颞叶结构的异常改变起到了至关重要的作用,并可为致痴灶的定位提供参考依据。     

成人积水性无脑畸形临床特点分析

目的探讨成人积水性无脑畸形的病因、临床表现及影像学改变。方法回顾性分析2例成人积水性无脑畸形患者的临床特点。结果患者临床表现特殊,肢体运动功能受损较轻,1例轻度智力发育迟滞,2例患者均能融入社会生活,言语功能仍然正常。头颅CT和MRI一侧大脑实质大部分消失,代以脑脊液样组织。1例头颅MRA双侧大脑中动脉未见显影。结论成人积水型无脑畸形临床表现相对轻微,提示可能与胎儿时期的皮层重建有关。     

50例新生儿缺血缺氧性脑病临床及CT分析

缺血缺氧性脑病（HIE）是围产期新生儿最常见的中枢神经系统病变，不仅严重威胁新生儿的生命健康，而且是伤残儿童最常见的病因之一．我院儿科于1988年-1994年6月共收治新生地缺血缺氧性脑病50例，其中25例做了脑脊液检查，全部病例做了头颅CT检查·现就其临床及CT结果分析报告如下。资料与方法一、HIE诊断标准[1-5]：（1）有宫内或生后窒息（Arrar评分1分钟wt3分·5分钟wt5分）或有引起缺血缺氧性的严重原发病；（2）有意识障碍及肌张力改变持续在生后24小时以上；（3）有惊厥或呼呗节律不整及脑水肿表现；（4）CT呈现2个区域（脑叶…     

癫癎痴笑发作8例临床分析

目的总结癫癎痴笑发作（GS）的病因、临床特点和临床进展，为制定临床诊疗决策提供依据。方法：2012年1月～2013年12月，结合患者头颅MRI、PET—CT和24 h视频脑电图（V-EEG）检查结果及典型GS的临床表现，收集8例门诊诊断为GS的病例进行分析。结果：根据患者的临床表现和辅助检查综合判断，6例诊断为症状性GS，2例隐原性GS。其中3例仅有痴笑发作，5例合并有1～2种其他发作类型。所有患者均接受1～3种抗癫癎药治疗，结果3例发作得到控制，2例发作减少〉50％，3例无效。结论：通过分析病历，进一步强调了除下丘脑错构瘤为常见病因外，额、颞、顶、枕等脑叶的病变也可导致GS。对MRI正常的患者，PET—CT能更好地显示病灶。对于EEG和MRI均正常的患者，典型的临床症状和诊断性治疗更有助于早期确诊及改善预后。     

宫内窒息胎儿脐血S100β蛋白的变化

胎儿宫内窒息是新生儿缺血、缺氧性脑病的主要诱因。对缺血缺氧性脑病的诊断仍以临床表现及CT、B超、脑电图为诊断依据，缺乏生化诊断指标。S100β是近年发现的代表神经系统损伤的蛋白。有研究认为，可作为脑损伤及损伤程度的指标。     

新生儿高胆红素血症MRI检查临床分析

目的探讨磁共振成像（MRI）技术协助诊断胆红素脑病的临床价值。方法回顾性分析2012年1月-2012年12月期间,对本院14例高胆红素血症患儿行头颅MRI检查的临床资料。结果高胆红素血症14例中,有胆红素脑病早期临床表现的5例,有痉挛期临床表现的1例,MRI异常者有5例（去除可疑1例）,均显示双侧苍白球T1WI信号及T2-FLAIR（抑水系列）呈对称性高信号,在T2WI呈正常信号,脑干听觉诱发电位（BAEP）异常有7例。结论 MRI能为早期高胆红素脑病提供客观指标,尤其对临床可疑病例的诊断方面有较高的临床价值。     

急性病毒性脑炎患者癫痫发作症状42例分析

目的：探讨急性病毒性脑炎患者癫痫发作症状的临床特点、影像学、脑电图（EEG）改变及抗痫药物疗效。方法：回顾性分析42例急性病毒性脑炎患者癫痫发作症状患者的临床特点、影像学、视频脑电图（V—EEG）改变及抗痫药物的治疗。结果：42例急性病毒性脑炎患者癫痫发作症状,以部分性发作为主要发作形式,以颞叶癫痫症状多见,脑部磁共振（MRI）主要表现为局灶性炎性水肿、或合并出血等破坏性病灶,V-EEG以慢波改变及局灶性痫样放电为主。结论：患者的临床表现、影像学、V-EEG改变对病毒性脑炎患者癫痫发作症状的诊断、治疗及预后评价均具有重要作用,临床需个体化选择抗痫药进行有效治疗。     

Posterior reversible encephalopathy syndrome in a child with steroid-resistant nephrotic syndrome: a case report and review of literature

Posterior reversible encephalopathy syndrome (PRES) is a rare and serious syndrome of central nervous system that can develop in both adults and children. It is characterized by acute onset of headache, confusion, seizures or focal neurological deficits along with radiological findings of white matter abnormalities in the parietal and occipital lobes. In the past ten years, this syndrome has been described mainly in adults, rare in children. Here, we report a case of PRES presenting in a 12-year-old girl with steroid-resistant nephrotic syndrome. Her neurological symptom was rapidly recovered after control of hypertension without discontinuation of cyclosporine A.     

Neuropsychological deficits in a case of H2S anoxic encephalopathy.

A comprehensive examination of the neuropsychological status of a young male patient of anoxic encephalopathy due to acute hydrogen sulfide intoxication was made. Agraphia, acalculia, deficits of associative verbal fluency and design fluency, and general intellectual deterioration were noted. Qualitative analysis of number operation revealed a specific impairment in the performance of division. Further analysis of graphic output showed a phonological substitution feature. The disorder of written language in the absence of disorders of language and communication in other modalities and praxis can be considered pure agraphia. The possible neural substrate for pure agraphia and partial acalculia was also discussed.     

Severe form of encephalopathy associated with 2009 pandemic influenza A (H1N1) in Japan

BackgroundEvery year, an estimated 200–500 children in Japan develop influenza-associated encephalopathy (IAE), and 10–30% of these children die.ObjectiveTo clarify the clinical features of a severe form of acute encephalopathy seen with 2009 pandemic influenza A (H1N1).Study designThis retrospective survey examined 20 children with acute encephalopathy associated with the 2009 pandemic influenza A (H1N1) who died or were in a prolonged deep coma with a flat electroencephalogram tracing and loss of spontaneous respiration. We obtained demographic, clinical, laboratory, and neuroimaging data through interviews with the attending physicians and chart reviews.ResultsSubjects were 13 boys and seven girls. Their median age was 45 (range 11–200) months. Five patients had one or more pre-existing conditions. Acute encephalopathy developed within 2 days after influenza onset in 16 patients. As the initial neurological symptom, delirious behavior was seen in six children, and brief seizures in six. Eighteen patients were comatose within 6 h of the onset of encephalopathy. Marked brain edema on computed tomography (CT) was seen in all but one patient. Brainstem lesions on CT were recognized in 12 patients. Sixteen patients died 0–45 (median 2.5) days after the onset of acute encephalopathy, and the others remained in deep comas without spontaneous respiration.ConclusionsThe clinical course of the patients was characterized by an onset with mild neurological symptoms and rapid deterioration of consciousness into coma. Head CT revealed marked cerebral edema, often associated with brainstem lesions.     

Expanding the clinical spectrum of mitochondrial 3‐hydroxy‐3‐methylglutaryl‐CoA synthase deficiency with Turkish cases harboring novel HMGCS2 gene mutations and literature review

Mitochondrial 3‐hydroxy‐3‐methylglutaryl‐CoA synthase (mHS) deficiency is a very rare autosomal recessive inborn error of ketone body synthesis and presents with hypoketotic hypoglycemia, metabolic acidosis, lethargy, encephalopathy, and hepatomegaly with fatty liver precipitated by catabolic stress. We report acute presentation of two patients from unrelated two families with novel homozygous c.862C>T and c.725‐2A>C mutations, respectively, in HMGCS2 gene. Affected patients had severe hypoketotic hypoglycemia, lethargy, encephalopathy, severe metabolic and lactic acidosis and hepatomegaly after infections. Surprisingly, molecular screening of the second family showed more affected patients without clinical findings. These cases expand the clinic spectrum of this extremely rare disease.     

Local shwartzman reaction in the rectal mucosa in acute diarrhoea

A microvascular lesion characterized by endothelial and platelet damage leading to intravascular coagulation, thrombosis and vascular dehiscence with haemorrhage was found in capillaries and venules in the rectal mucosal lamina propria of adults with acute diarrhoea. The lesion morphologically resembled the local Shwartzman reaction. There was no correlation between the prevalence of the vascular lesion and the presence of recognized enteric pathogens, but there was a significant correlation with the clinical severity of the illness. It is suggested that this vascular lesion could be the result of the failure of the epithelioluminal barrier in the colon and it may also be one of the determinants of the clinical severity of illness in adults with acute diarrhoea.     

慢性苯妥英钠中毒性脑病1例并文献复习

目的:探讨苯妥英钠(phenytoin,PHT)中毒性脑病的临床特点及诊断治疗要点,以提高神经科医生对该病的认识.方法:对1例慢性PHT中毒性脑病患者的临床资料、病因、诊断、鉴别诊断及治疗结合文献进行回顾性分析.结果:此例患者最初被诊断为缺血性脑血管病,予改善循环等治疗后未见好转;经详细追问病史、监测血药浓度、腰椎穿刺、脑电图、调整治疗方案后患者明显好转,出院.结论:PHT中毒后临床表现复杂多样,有些不易与脑干或小脑梗死相鉴别,容易误诊,应提高对其的认识,从而实现早期诊断和治疗.     

Crimean-Congo haemorrhagic fever presenting as epididymo-orchitis

BackgroundCrimean-Congo haemorrhagic fever (CCHF) is a potentially fatal disease caused by a tick-borne virus.ObjectivesA 53-year-old man presented with fever and acute painful scrotal swelling simulating acute epididymo-orchitis.Study designBased on the clinical and epidemiological findings, CCHF virus infection and epididymo-orchitis were suspected. This symptom, rarely reported in viral haemorrhagic fevers, was observed in this case.ResultsThe diagnosis was confirmed by detection of the IgM antibody to CCHF virus and positive RT-PCR.ConclusionWe report the first case of imported CCHF presenting as epididymo-orchitis. This symptom is a rare complication of CCHF, and the clinician should consider this entity in the differential diagnosis of adults with epididymo-orchitis.     

Childhood attention deficit/hyperactivity disorder in adults with anxiety disorders.

BACKGROUND: Previous research has reported co-morbidity between attention deficit hyperactivity disorder (ADHD) and anxiety disorders. Interpretation of these findings is complicated by symptom overlap in the clinical presentation of the disorders. We estimate the prevalence of ADHD in both the current and childhood histories of adults with anxiety disorders, while taking symptom overlap into account. We also evaluate the utility of the Wender Utah Rating Scale (WURS) for retrospective reporting of ADHD. METHODS: Consecutive admissions (N = 149) to an anxiety disorders clinic were given a diagnostic and psychometric assessment. The WURS was administered to obtain a retrospective diagnosis of childhood ADHD. Twenty-nine of the 35 people surpassing the cut-off score on the WURS were given a structured interview of adult ADHD symptoms. RESULTS: The WURS contains many 'internalizing' items that may inflate retrospective accounts of ADHD. After taking this into account, there is still a significantly higher prevalence of ADHD in the retrospective reports of adults with anxiety disorders (15%) than would be expected by chance (4%). Furthermore, of those who meet retrospective criteria for ADHD, 45% (13 of 29) continue to meet diagnostic criteria for ADHD as adults. CONCLUSIONS: The WURS may require considerable revision for use with clinical populations. In spite of these difficulties with retrospective assessment, available evidence indicates that ADHD is more prevalent in the histories of anxiety disordered patients than would be expected from base rates.     

Prognostic implications of cytology in acute leukemia in the adult: The case for subacute leukemia

One hundred sixty-nine cases of acute leukemia in adults have been prospectively classified on the basis of the original peripheral blood smear and bone marrow into two subclasses of acute lymphatic leukemia (acute lymphocytic leukemia and subacute lymphocytic leukemia) and five subclasses of acute nonlymphocytic leukemia (acute myelogenous leukemia, subacute myelogenous leukemia, acute myelomonocytic leukemia, erythroleukemia, and acute promyelocytic leukemia). A number od differences in presenting symptoms and physical findings were found among the various subclasses of acute leukemia. Most significant, however, was the marked difference in median survival between patients with acute lymphocytic leukemia (10 months) and subacute lymphocytic leukemia (16.5 months; p=0.01) and acute myelogenous leukemia (2 months) and subacute myelogenous leukemia (6 months; p0.01). The division on the basis of morphology of the acute leukemias into acute and subacute forms has important prognostic significance in terms of survival and perhaps also in terms of response to therapy.     

低氧预处理对大鼠海马神经元缺氧耐受性和bcl-2表达的影响

采用免疫组织化学方法观察了低氧预处理对大鼠海马神经元缺氧耐受性和 bcl-2表达的影响。结果显示 ,经低氧预处理的海马神经元缺氧 -复氧后 bcl-2表达较对照组明显增强 ,神经元损伤程度减轻 ,神经元存活数明显高于对照组。本结果表明 ,低氧预处理可使海马培养神经元对缺氧产生耐受 ,增加缺氧 -复氧后神经元 bcl-2的表达。提高神经元存活数