Reye's syndrome: a clinical review.

Reye''s syndrome is a virus-associated biphasic disease that causes acute encephalopathy in infants and children. Epidemiologic and experimental data support the hypothesis that it is a multifactorial disease of modern civilization. Just as young patients seem to be recovering uneventfully from the first phase of the illness, usually a nonspecific viral-like illness such as a respiratory tract infection or gastroenteritis, the second phase, encephalopathy, starts unexpectedly, with vomiting and sensorial changes. Identifying the syndrome early ;in the second phase and referring the child to a specialized centre with the experience, staff and facilities to manage this phase has improved the numbers and neurologic condition of survivors, though the overall mortality is still about 20%. Therapy is primarily directed at facilitating adequate cerebral perfusion pressure.     

Defects of metabolism of fatty acids in the sudden infant death syndrome.

Two hundred consecutive cases of the sudden infant death syndrome were reviewed for the presence of fat in the liver; 14 showed diffuse panlobular microvesicular fatty change indistinguishable from that found in Reye''s syndrome. Samples of frozen liver were available in five of the 14 cases; histochemical analysis showed well preserved cytochrome oxidase and succinate dehydrogenase activity in all five, uncharacteristic of Reye''s syndrome. Fatty acyl-coenzyme A dehydrogenase activity in the liver was assayed biochemically in two of the same five cases with severe hepatic fatty infiltration; both showed a defect in medium chain acyl-coenzyme A dehydrogenase activity using the substrate octanoyl-coenzyme A. Both cases also showed cerebral oedema in association with fatty infiltration of renal tubules, myocardium, and skeletal muscle, characteristic of Reye''s syndrome. It is concluded that diffuse panlobular microvesicular fatty change of the liver in victims of the sudden infant death syndrome, although essentially non-specific, indicates that the state of mitochondrial enzymes should be investigated.     

Reye's Syndrome

Reye''s syndrome (encephalopathy with fatty infiltration of the viscera) is an acute illness of childhood that produces hepatic dysfunction and metabolic encephalopathy. The disease is fatal in as many as 40% of cases. The cause is unknown. Several environmental agents, particularly salicylates and aflatoxin, have been implicated as possible toxins in this disorder. Treatment is directed at controlling intracranial pressure, reversing metabolic abnormalities and providing intensive supportive care. Normal neurologic function returns in most survivors.     

Is a compromised interferon response an etiologic factor in Reye's syndrome?

Young mice injected with sublethal doses of Toximul MP8, a typical commercial polyoxyethylene ether-based emulsifier, died more frequently when infected with encephalomyocarditis virus than did control mice. Lymphocytes taken from emulsifier-injected mice responded poorly to interferon induction, unlike lymphocytes from control animals. Interferon protected control mice against viral encephalomyocarditis, but such protection was not equally demonstrable in emulsifier-injected mice. These data suggest that the enhanced lethality of encephalomyocarditis virus in emulsifier-injected mice is associated with and perhaps caused by a compromised interferon response in these animals. Since these emulsifiers are commonly found in the environment in areas where forests are sprayed with pesticides, a group of children suffering from Reye''s syndrome who lived in such areas was investigated. Blood samples were obtained from five children with influenza B-associated Reye''s syndrome during their acute illness and during convalescence. Lymphocytes obtained from these samples and from peripheral blood samples from healthy children (controls) were induced to synthesize interferon by exposure to Newcastle disease virus. The lymphocytes from the convalescent patients and from the controls responded well to induction. However, the lymphocytes obtained from patients and from the controls responded well to induction. However, the lymphocytes obtained from patients during the acute phase of Reye''s syndrome responded very poorly and produced significantly less interferon.     

Reye's syndrome in a newborn infant

A case of Reye''s syndrome in a newborn infant, believed to be the first recorded in the perinatal period, is reported. The clinical, biochemical and histological findings are those previously recognized in older infants. The intractable acidosis, hypoglycemia and extreme degree of lactic acidosis reflect an advanced degree of hepatic involvement, probably initiated in utero, with resultant impairment of glycogenolysis and intermediary metabolism.     

SPECT 对症状性颅内动脉狭窄支架成形术的疗效评价

目的:通过SPECT脑血流灌注显像,对症状性颅内动脉粥样硬化性狭窄行颅内支架成形术,进行血流动力学的疗效评价。方法:对42例症状性颅内动脉粥样硬化性狭窄(狭窄程度>50%)患者,术前通过SPECT,分为低灌注组和正常灌注组。术后3月复查SPECT,做出血流动力学的疗效评价。通过术前及术后1年mRS评分,比较两组的远期疗效。结果:低灌注组发病症状一般为缺血性卒中,正常灌注组一般为TIA(P<0.01)。低灌注组术后血流灌注较术前改善明显(P<0.01)。正常灌注组复发性TIA的发生率较低灌注组高(P<0.05)。低灌注组术前术后mRS评分有统计学差异(P<0.05)。结论:颅内支架成形术可以明确改善颅内低灌注区的血流灌注,对低灌注组术后缺血事件的预防效果好,并有改善神经功能作用。因此,存在颅内低灌注区的患者更具有颅内支架成形术的手术适应症。     

Subarachnoid haemorrhage: older patients have low cerebral blood flow.

Daily estimations of hemispheral cerebral blood flow using the xenon-133 inhalation technique was made in 116 patients during the first three weeks after subarachnoid haemorrhage. The patients'' cerebral perfusion on average remained less than the normal perfusion expected for their age (based on a single estimation of cerebral blood flow in 67 volunteers). On each separate day after subarachnoid haemorrhage cerebral blood flow was inversely related to the patient''s age. Older patients seem especially at risk of developing cerebral ischaemia after subarachnoid haemorrhage. The clinical outcome was more often unfavourable in older patients--that is, in those who tended to have the lowest cerebral blood flow. Present results support the view that episodes of low cerebral blood flow lead to a poor outcome after subarachnoid haemorrhage. Because of the risk of inducing cerebral ischaemia great care should be exercised by physicians administering hypotensive drugs to older patients after subarachnoid haemorrhage.     

Cerebral hemodynamics during microgravity.

As one of the causes of the space adaptation syndrome, an increased intracranial pressure due to the cephalad fluid shift is suggested. In the present study, we measured intracranial pressure (ICP), aortic pressure and cerebral flow velocity (CFV) in anesthetized rats (n=5) during 4.5 sec of microgravity induced by free drop. The rats were set at horizontal prone (Flat) and 30-degree head-up whole body tilting (HU) positions to examine the effect of gravitational pressure gradient. Then, arterial pressure at the eye level (APeye), cerebral perfusion pressure (CPP; CPP=APeye-ICP), and CPP-CFV relationship was calculated. In HU position, ICP, APeye, and CPP increased by 2.2 +/- 0.4, 12.3 +/- 2.0, and 10.1 +/- 1.7 mmHg respectively. However, CFV did not change significantly. In Flat position, none of these variables did not change significantly. In HU position the slope of CPP-CFV relationship decreased, suggesting the increased cerebral flow resistance. However, it did not change in Flat position. These results can be understood by the disappearance of gravitational pressure gradient by microgravity and the cerebral autoregulation.     

重型颅脑损伤后颅内高压的治疗研究进展

重型颅脑损伤后颅内压增高预示着不良的神经功能预后和极高的死亡率,一直是临床治疗中的研究热点,可采取高渗性脱水,亚低温疗法,巴比妥昏迷治疗及外科手术干预等治疗措施控制颅内压。由于亚低温治疗会增加患者发生肺炎的风险,巴比妥类药物副作用较大,现均已少用。近来研究发现,监测颅内压、脑灌注压、脑组织氧分压并指导临床治疗,可降低死亡率与改善预后。也有研究发现去骨瓣减压术治疗顽固性颅内高压与神经功能预后较差有关。目前关于颅内高压治疗的最佳方案仍存在争议,未来还需根据患者病情,为其制定规范化与个体化的治疗方案,预防继发性颅脑损伤,降低颅内压。本文就近年来重型颅脑损伤后颅内高压的治疗进展进行阐述。     

Predictive value of SS-B precipitating antibodies in Sjo?gren's syndrome.

As part of a screening programme several patients were identified with antibodies to the nuclear antigen SS-B. Fifteen were examined and 11 found to have Sjögren''s syndrome, though this had not been suspected by most of the referring physicians. In contrast, among a group of 17 patients with overt Sjögren''s syndrome, most of whom also had rheumatoid arthritis, only one had antibodies to SS-B. Patients presenting with polyarthralgia found to be SS-B positive may be likely to develop Sjögren''s syndrome but unlikely to develop rheumatoid arthritis. The detection of SS-B antibodies may antedate clinical evidence of Sjögren''s syndrome by months or even years. These results emphasise the clinical heterogeneity of Sjögren''s syndrome.     

Effect of an oral serotonin antagonist,ketanserin, on plasma ACTH concentrations in Nelson's syndrome.

A study was performed to see whether ketanserin, a serotonin antagonist, would reduce the raised concentrations of adrenocorticotrophic hormone (ACTH) in patients with Nelson''s syndrome. Six patients who had undergone bilateral adrenalectomy for Cushing''s disease and who had Nelson''s syndrome were given ketanserin 40 mg twice daily and placebo, for at least two months each, in a double blind crossover study. Ketanserin had no effect on ACTH concentrations. In healthy people serotonin seems to have a stimulatory role in the regulation of ACTH secretion, and the effect of ketanserin in reducing the ACTH response to hypoglycaemia suggested that it might prove useful in Nelson''s syndrome. These results show that it is not indicated in these patients.     

无创颅内压监测在创伤性急性弥漫性脑肿胀治疗中的临床应用研究

目的:探讨无创颅内压监测(Intracranial pressure,ICP)监测对救治创伤性急性弥漫性脑肿胀(Posttraumatic acute diffuse brains welling,PADBS)患者的辅助作用,为无创ICP检测的临床应用提供参考依据。方法:收集2011年1月至2016年1月我院神经外科收治的114例PADBS患者的临床资料进行回顾性分析,依据纳入与排除标准共取得病例组53例,对照组61例,分别给予采用无创颅内压监测和有创颅内压监测,根据患者资料进行组间比较及生存分析。结果:病例组与对照组不同分型颅内压检测值比较无统计学差异(P0.05);患者48h内进行大骨瓣开颅手术时间的生存分析,病例组中位手术时间为35.6个月,对照组中位DFS为33.5个月,两组患者检测进行手术时间的生存曲线无统计学差异(P0.05);有创ICP检测患者继发性出血率为3.2%,脑脊液感染病况为7.9%;两组患者预后情况差异并无统计学意义(P0.05)。无创ICP检测患者住院天数短于有创ICP检测患者(P0.05)。结论:无创ICP应用于PADBS治疗的临床价值与有创ICP相当,但无创ICP更便捷、创伤小,有利于对患者进行持续性检测。     

利多卡因联合尼莫地平预防颅内动脉瘤术后脑血管痉挛的临床疗效观察

目的:比较利多卡因加尼莫地平联合治疗与尼莫地平单药治疗在预防颅内动脉瘤术后脑血管痉挛(CVS)的临床疗效与患者预后评估。方法:选择2012年1月-2015年6月在我院就诊的颅内动脉瘤患者65例,通过显微外科开颅动脉瘤夹闭术后,分成对照组与治疗组。对照组接受常规尼莫地平治疗,治疗组在尼莫地平治疗基础上联合利多卡因治疗,观察比较两组患者在14天内的大脑中动脉血流速度变化,并分析两组患者发生CVS、脑梗死、临床死亡情况及患者预后差异。结果:治疗组的大脑中动脉血流速度在治疗前,治疗后第7天,治疗后第14天分别为133.81±12.35m/s、78.88±5.05m/s、77.28±4.78m/s明显低于对照组的136.02±11.67m/s、96.74±4.25m/s、85.96±7.87m/s,差异有统计学意义(P=0.000)。治疗组患者最终发生CVS3例,对照组9例,差异有统计学差异(P=0.026)。但在脑梗死例数、死亡例数及最终预后上,未发现明显差异。结论:尼莫地平联合利多卡因治疗预防颅内动脉瘤夹闭术后CVS的临床效果显著优于尼莫地平单药组,可以作为术后预防有效的治疗联合方案。     

Cerebral autoregulation is preserved in postural tachycardia syndrome.

To test whether cerebral autoregulation is impaired in patients with postural tachycardia syndrome (POTS), we evaluated 17 healthy control subjects and 27 patients with POTS. Blood pressure, heart rate, and cerebral blood velocity (transcranial Doppler) were recorded at rest and during 80 degree head-up tilt (HUT). Static cerebral autoregulation, as assessed from the change in cerebrovascular resistance during HUT, was the same in POTS and in controls. The properties of dynamic cerebral autoregulation were inferred from transfer gain, coherence, and phase of the relationship between blood pressure and cerebral blood velocity estimated from filtered data segments (0.02-0.8 Hz). Dynamic cerebral autoregulation of patients with POTS did not differ from that of controls. The patients' dynamic cerebral autoregulation did not change over the course of HUT, despite increased tachycardia suggestive of worsening orthostatic stress. Inflation of military anti-shock trouser pants substantially reduced the tachycardia of patients with POTS without affecting cerebral autoregulation. Symptoms of orthostatic intolerance were reduced in one-half of the patients following military anti-shock trouser pants inflation. We conclude that cerebral perfusion and autoregulation in many patients with POTS do not differ from that of normal control subjects.     

早期康复护理对肠内营养治疗的高血压颅内出血 患者预后的影响

目的：研究早期康复护理对肠内营养治疗的高血压颅内出血患者预后的影响。方法：选取2010 年1 月~2012 年10 月间入 院诊治的高血压颅内出血并应用肠内营养支持治疗的患者120 例,随机分为实验组（60 例）和对照组（60 例）。对照组应用常规护 理办法,实验组在其基础上应用早期康复护理,随访1 年后观察两组患者预后的生活质量情况、GCS（格拉斯哥昏迷指数）评分以 及并发症的发生情况。结果：随访1 年后,实验组的生活质量显著高于对照组（P<0.05）;护理干预前两组患者的GCS 评分无差异, 护理干预后实验组的GCS 评分显著高于对照组（P<0.05）;实验组在肺部感染、下肢静脉血栓、肩手综合征等并发症的发生例数均 显著低于对照组（P<0.05）。结论：针对肠内营养支持治疗的高血压颅内出血患者应用早期康复护理可有效改善患者的昏迷程度、 预防并发症,有助于提高患者的生活质量。     

颈动脉支架置入术后患者认知功能的变化及CT灌注成像技术的疗效评估价值研究

目的:探讨颈动脉狭窄患者在行颈动脉支架置入术后认知功能的变化情况,并分析CT灌注成像(CTP)对手术疗效的评估价值。方法:选取2015年10月到2018年3月在济宁医学院附属日照市人民医院接受治疗的颈动脉狭窄患者80例,其中有症状性颈动脉狭窄患者49例作为有症状组,无症状性颈动脉狭窄患者31例作为无症状组。所有患者均接受颈动脉支架置入手术及CTP检查,采用蒙特利尔认知评估量表(MoCA)、简易智能精神状况量表(MMSE)、搭火柴测验(Stick Test)综合评价颈动脉狭窄患者的术前、术后1周、术后3个月、术后6个月认知功能的变化情况,比较有症状组和无症状组患者的CTP相对灌注参数。结果:术后1周,颈动脉狭窄患者的MoCA总分、视空间/执行能力、注意力、延迟回忆以及MMSE总分、Stick Test总分较术前有所降低(P0.05);术后3个月、术后6个月,颈动脉狭窄患者的MoCA总分、视空间/执行能力、注意力、延迟回忆以及MMSE总分、Stick Test总分较术前有所升高(P0.05)。术前,有症状组的相对血流达峰时间、相对平均通过时间长于无症状组,相对脑血流量低于无症状组(P0.05);术后1周,无症状组的相对血流达峰时间较术前有所缩短,且短于有症状组(P0.05);术后1周,有症状组的相对血流达峰时间、相对平均通过时间较术前有所缩短,相对脑血流量较术前有所升高(P0.05);两组术前、术后1周相对脑血容量比较均无统计学差异(P0.05)。结论:颈动脉支架置入术后患者会出现暂时的、可逆的认知功能恶化,但最终认知功能会得到明显的改善。CTP可发现异常的脑灌注情况,同时能够较好地评价颈动脉支架置入术治疗颈动脉狭窄患者的疗效。     

Towards a more rapid diagnosis of rapidly progressive glomerulonephritis.

An attempt was made to provide simple practical guidelines to alert general practitioners to the diagnosis of rapidly progressive glomerulonephritis and lead to early referral to hospital. The duration of illness before referral to this hospital and its effect on outcome in patients with crescentic nephritis were assessed retrospectively from the case notes of 24 patients referred over two years. Four patients had Goodpasture''s syndrome, 11 Wegener''s granulomatosis, seven microscopic polyarteritis, and two idiopathic progressive glomerulonephritis. The duration of symptoms before referral to the local hospital was similar in the four groups of patients and varied from one week to 28 months (mean 10 months). The duration of stay in the local hospital was two, nine, 11, and 180 days in the patients with Goodpasture''s syndrome and a mean of four days (range one to eight) in those with Wegener''s granulomatosis and 10 days (one to 18 days) in those with microscopic polyarteritis. In the local hospital the diagnosis was based on the results of renal biopsy and detection of antibodies to glomerular basement membrane in two patients with Goodpasture''s syndrome and on the results of renal biopsy in seven of the other patients aided by the detection of antibodies to the cytoplasm of neutrophils (ANCA) in 10. Three of the 24 patients died and four required maintenance haemodialysis. Patients who present to their general practitioners with persistent non-specific symptoms should have a urine dipstick test and then blood tests and emergency referral to hospital if necessary. Hospital physicians should be aware of the speed and accuracy with which current assays can confirm a diagnosis of rapidly progressive glomerulonephritis.     

Hemodynamic Factors May Play a Critical Role in Neurological Deterioration Occurring within 72 hrs after Lacunar Stroke

Background

Whether a perfusion defect exists in lacunar infarct and whether it is related to early neurological deterioration (END) is still under debate. The aim of this study was to evaluate whether END in lacunar infarct is related to a perfusion defect using diffusion-weighted imaging (DWI), diffusion tensor imaging (DTI) and perfusion MR imaging.

Methods

One hundred and forty-one consecutive patients had an MRI scan within 30 hours after onset of symptoms and 43 patients with acute lacunar infarct and classic lacunar syndrome were recruited. The MRI sequences included DWI, DTI and cerebral blood flow (CBF) maps to respectively represent the topographic locations of acute infarcts, the corticospinal tract and perfusion defects. The END was defined in reference to the National Institute of Health Stroke Scale (NIHSS) as an increase ≧2 within 72 hours. Cohen''s Kappa coefficient was used to examine the reliability between the 2 image readers. A multivariate logistic regression model was constructed adjusting for baseline variables.

Results

Ten out of the 43 patients had END. Patients having END was significantly associated with lower chances of favorable and good outcomes at 3 months (p = 0.01 and p = 0.002, respectively). END was predicted when the non-core hypoperfused area overlapped on the corticospinal tract, which is defined as the expected END profile. Cohen''s Kappa coefficient between the 2 image readers to define images of expected END profiles was 0.74. In 15 patients with expected END profile, 9 had END clinically, whereas 28 patients had no expected END profile, and only 1 patient had END (p<0.0001). After adjusting for sex, the expected END profile was still associated with END (odds ratio, 42.2; p = 0.002).

Conclusion

Our study demonstrated that the END in acute lacunar stroke is likely related to the transformation of non-core hypoperfused area into infarction in the anatomy of corticospinal tracts.     

The role of perfusion computed tomography in the prediction of cerebral hyperperfusion syndrome

Background

Hyperperfusion syndrome (HPS) following carotid angioplasty with stenting (CAS) is associated with significant morbidity and mortality. At present, there are no reliable parameters to predict HPS. The aim of this study was to clarify whether perfusion computed tomography (CT) is a feasible and reliable tool in predicting HPS after CAS.

Methodology/Principal Findings

We performed a retrospective case-control study of 54 patients (11 HPS patients and 43 non-HPS) with unilateral severe stenosis of the carotid artery who underwent CAS. We compared the prevalence of vascular risk factors and perfusion CT parameters including regional cerebral blood volume (rCBV), regional cerebral blood flow (rCBF), and time to peak (TTP) within seven days prior to CAS. Demographic information, risk factors for atherosclerosis, and perfusion CT parameters were evaluated by multivariable logistic regression analysis. The rCBV index was calculated as [(ipsilateral rCBV - contralateral rCBV)/contralateral rCBV], and indices of rCBF and TTP were similarly calculated. We found that eleven patients had HPS, including five with intracranial hemorrhages (ICHs) of whom three died. After a comparison with non-HPS control subjects, independent predictors of HPS included the severity of ipsilateral carotid artery stenosis, 3-hour mean systolic blood pressure (3 h SBP) after CAS, pre-stenting rCBV index >0.15 and TTP index >0.22.

Conclusions/Significance

The combination of severe ipsilateral carotid stenosis, 3 h SBP after CAS, rCBV index and TTP index provides a potential screening tool for predicting HPS in patients with unilateral carotid stenosis receiving CAS. In addition, adequate management of post-stenting blood pressure is the most important treatable factor in preventing HPS in these high risk patients.     

Benign Intracranial Hypertension following Corticosteroid Withdrawal in Childhood

In an 18-month period seven children who were treated for a variety of neurological and non-neurological diseases, and in whom the corticosteroid or corticotrophin dosage was reduced, developed a syndrome indistinguishable from “benign intracranial hypertension.” The total duration and rate of reduction of corticosteroid dosage and perhaps an underlying susceptibility to cerebral oedema appear to be important factors in this syndrome. Usually patients can be treated without the need for special neuroradiological studies. Possibly mild forms of this condition are not uncommon, but its pathogenesis is still uncertain.