Neuroimaging and spectroscopy in children with epileptic encephalopathies

AIMS: To investigate the nature of the unifocal cortical abnormalities on FDG positron emission tomography (PET) in children with an epileptic encephalopathy but no focal abnormality on electroencephalogram or standard magnetic resonance imaging (MRI). METHODS: Repeat FDG PET, surface rendered high resolution MRI, and single voxel magnetic resonance proton spectroscopy of the areas of abnormal metabolism compared to the contralateral side in 11 children aged 2 to 12 years. Imaging was repeated after a median of 13 months. RESULTS: Visual analysis of repeat FDG PET revealed similar abnormalities in 10 of 11 children. Semiquantitative analysis revealed similar sited abnormalities in eight children. One child with ictal hypermetabolism initially had an inconsistent second scan. Magnetic resonance spectra in three children showed the N-acetyl-aspartate/choline ratio was lower in the hypometabolic focus than in the reciprocal area on the opposite side, in two children it was higher, and in one child it was equal. Surface rendered MRI was normal in seven of eight children, and showed temporal lobe asymmetry in one. CONCLUSION: In children with established epileptic encephalopathies most hypometabolic areas on FDG PET are stable over time. While focal neuronal loss is likely in these areas in some children, microdysplasias or other focal cortical dysplasias are probable in others.     

The changing clinical pattern of Reye's syndrome 1982-1990

OBJECTIVE: To describe trends in the clinical pattern of Reye's syndrome in the British Isles between 1982 and 1990; and to determine the relation between any changes and the June 1986 warnings against the use of aspirin in children. DESIGN: Development, and application to reported cases, of a scoring system designed such that patients showing the typical clinical and pathological features of 'classical' Reye's syndrome scored highly. The relations between 'Reye scores' and a number of explanatory variables were explored using multivariable analysis. SETTING: British Isles. SUBJECTS: 445 cases fulfilling the Reye's syndrome case definition reported to the surveillance scheme between January 1982 and December 1990. MAIN OUTCOME MEASURE: Individual 'Reye score'. RESULTS: Cases with high scores were more likely to have occurred in the 4 1/2 year period before June 1986 compared with the subsequent period (p < 0.006). Numbers of cases in the low and intermediate score categories declined by about 50% after June 1986, whereas those in the high category fell by 79%. High scorers were more likely to have received aspirin (p < 0.0001) and were older than intermediate and low scorers (p < 0.008). No relation was identified between score and season of onset. CONCLUSIONS: The decline in Reye's syndrome after the aspirin warnings cannot be explained entirely, as has been proposed, by improved diagnosis of 'Reye-like' inherited metabolic and other disorders: this would not account for the greater decline of the high scoring subgroup which also contained those cases most likely to resemble 'classical' Reye's syndrome and to have received aspirin. This study provides further evidence for the role of aspirin in a subset of cases meeting the standard diagnostic criteria for Reye's syndrome and supports the need to consider this disorder as a heterogeneous group of conditions including Reye-like inherited metabolic disorders.     

Felty's syndrome without rheumatoid arthritis?

Felty's syndrome (FS) is characterized by neutropenia and splenomegaly in patients with seropositive (RF+, anti-CCP+) rheumatoid arthritis (RA). As a result of neutropenia, affected persons are increasingly susceptible to infections. In the majority of patients, FS appears in the course of long-standing and well-established RA. Manifestations of FS without clinical but only with laboratory features of RA are extremely rare. We present a case of severe neutropenia and mild splenomegaly in a patient with high titers of RF and anti-CCP. For 4 years, patient's neutropenia remained asymptomatic. The neutropenia reduction to agranulocytosis was followed by successful methotrexate–corticosteroid therapy. Efficacy of the standard anti-RA therapy confirmed autoimmune mechanism of the Felty's neutropenia. The most important lesion from our case is to recognize this condition in the range of autoimmune rheumatic diseases without delay. We reviewed literature with non-articular FS.     

Childhood discoid lupus erythematosus: report of five new cases and review of the literature

Discoid lupus erythematosus (DLE) is an uncommon disease in childhood. In this paper we present five new cases of childhood DLE. Two of them are identical twin brothers, who developed similar lesions during an interval of 5 years. This is in favour of the hypothesis that both genetic factors and somatic mutations, due to environmental factors, are implicated in the pathogenesis. A review of the English language literature is also presented. In order to have better epidemiological data on this disease, all cases of childhood DLE, including those published in non-English literature and those not yet published, should be placed together and analysed.     

Assessing diagnosis in heart failure: which features are any use?

We assessed the value of symptoms, past history, medications and signs in the evaluation of patients who might have heart failure secondary to left ventricular systolic dysfunction. An open-access echocardiography service was set up to help identify patients with left ventricular systolic dysfunction who might benefit from treatment with an angiotensin-converting-enzyme inhibitor. History and examination were recorded for each of these patients. The patients were divided into groups according to whether left ventricular systolic function was preserved or not and whether various clinical features were present or not. Of 259 consecutive patients studied, 41 had impairment of left ventricular systolic function as assessed by echocardiography. Past history of myocardial infarction and displaced apex beat were the best single predictors of left ventricular systolic dysfunction as assessed by echocardiography. The combination of past history of myocardial infarction and displaced apex had the best positive predictive value of all. Patients with such clinical features or combinations of clinical features may not need echocardiography, and where access to this resource is limited, it could be reserved for patients without such diagnostic features.      

Polyclonal antibodies against the NB1-bearing 58- to 64-kDa glycoprotein of human neutrophils do not identify an NB2-bearing molecule

The neutrophil-specific NB antigen system has been serologically characterized with human alloantisera. Two alleles, NB1 and NB2, have been described; however, there may be important quantitative or qualitative variation in the expression of NB1 and NB2. Human alloantibodies have been used to identify the 58- to 64-kDa glycoprotein (GP) on which NB1 antigen is located, but an NB2 antigen- bearing molecule has not yet been identified. To identify the NB2 molecule, human alloantibody to NB1 was used to isolate the 58- to 64- kDa NB1 GP, and rabbits were immunized with this GP. Two rabbit antisera were produced. Both antisera immunoblotted and immunoprecipitated the 58- to 64-kDa GP on which NB1 is located, but neither identified the molecule on which NB2 is located. The inability of two rabbit polyclonal antibodies specific for the NB1 molecule to react with the NB2-bearing molecule suggests that considerable differences may exist between these two molecules or that NB2 as currently defined is not related to NB1.     

健康管理对冠状动脉旁路移植术后患者的康复作用

目的:以生活质量及血压、血糖、血脂等为指标,调查健康管理对冠状动脉旁路移植术后患者的康复作用。方法:选择2004-08/2005-08入院行冠状动脉旁路移植术的患者368例,均同意接受指定量表评估,并且得到了同济大学医学伦理委员会批准。按随机数字表法分为健康管理组和对照组进行随访观察:①对照组术后采用传统康复方式,即术后带药,遵医嘱复查。患者多根据自己的实际情况选择复查时间和医院,患者主动,医生被动。②健康管理组术后有精心安排的康复计划和内容,包括运动疗法、心理护理、健康教育及血压、血糖、血脂等的管理及干预措施,有指定的社区医疗单位,有指定的医生为患者复查,医生主动,患者被动或主动。所有患者入院后及术后1年由专职医生进行生活质量调查,填写SF-36健康状况问卷(共包括有关生理及精神健康的8个方面,每个方面分值范围均为0～100分,分数越高说明生活质量越高);测量血压;抽血检测血糖、血脂等。结果:368例患者中有342例坚持随访,随访率92.9%,其中健康管理组183例,对照组159例。①坚持随访的两组患者入院时生活质量评分差异无显著性意义(P>0.05),术后1年,健康管理组患者的生活质量评分显著高于对照组[生理机能:80.34±16.33,76.19±15.47;生理职能:55.78±38.57,46.18±40.22;躯体疼痛:82.28±22.79,76.10±21.37;一般健康状况:66.45±21.03,52.32±20.16;精力:67.66±19.51,61.34±21.31;社会功能:78.43±25.22,71.12±24.29;情感职能:68.07±33.60,57.50±35.75;精神健康:81.26±12.24,75.36±15.29(t=2.2439～6.3363,P<0.05～0.01)]。②坚持随访的两组患者入院时异常血压、血糖、血脂的发生率差异无显著性意义(P>0.05),术后1年,健康管理组患者的异常血压、血糖、血脂发生率均显著低于对照组[9%,27%;6%,17%;9%,23%(χ2=9.2859～17.3320,P<0.01)]。结论:冠状动脉旁路移植术后实行健康管理能明显提高患者的生活质量,降低异常血压、血糖、血脂的发生率;社区医疗单位在冠状动脉旁路移植术后患者的康复中起到重要作用。     

The Antihypertensive and Metabolic Effects of Low and Conventional Dose Cyclopenthiazide in Type II Diabetics with Hypertension

The antihypertensive efficacy and metabolic effects of cyclopenthiazide125 µg were compared with cyclopenthiazide 500 µgin patients with non-insulin dependent diabetes and hypertensionin a double blind, randomized crossover study. After a 6-weekplacebo period 24 patients with non-insulin dependent diabetesmellitus, stabilized on diet or oral hypoglycaemic agents, whohad a mean diastolic blood pressure between 90 and 120 mmHgafter receiving placebo for 6 weeks were given 125 µgor 500 µg cyclopenthiazide for 12 weeks. Patients thenreceived placebo for a further 6-week period, following whichthey received the alternate treatment dosage for 12 weeks. Therewere no differences between doses in their antihypertensiveeffects. While 500 µg significantly reduced systolic anddiastolic blood pressures, only diastolic pressure was significantlyreduced by 125 µg from pre-treatment values. The higherdose of cyclopenthiazide had greater effects on measures ofdiabetic control than did the 125 µg dose and the risein blood glucose after 12 weeks' treatment with 500 µgwas significantly different from pre-treatment values. Cyclopenthiazide125 µg had significantly less effect on triglycerides,potassium and urate, than did 500 µg. Cyclopenthiazide500 µg resulted in a significant fall in serum potassiumfrom pre-treatment values. There were no intertreatment differencesin the other variables measured. Cyclopenthiazide 125 µgis as effective as 500 µg in reducing diastolic bloodpressure in mildly hypertensive non-insulin dependent diabeticpatients. The higher dose had more pronounced adverse effectson glucose control and serum concentrations of triglycerides,potassium and urate.     

Analysis of antibodies against components of the autonomic nervous system in diabetes mellitus

Antibodies to autonomic nervous system structures have previously been detected using a complement fixation immunofluorescence test in the sera of patients with insulin-dependent diabetes mellitus (IDDM) and non-insulin dependent diabetes mellitus (NIDDM). These antibodies might play a role in the aetiology of autonomic neuropathy. Sera from 45 IDDM, 40 NIDDM and 52 control subjects were tested by immunofluorescence for antibodies to human sympathetic ganglia, human adrenal medulla and rabbit vagus nerve. The use of human sympathetic ganglia was compared with rabbit tissue for the detection of sympathetic ganglia antibodies; the results for these autonomic nervous system antibodies were also compared with results using an ELISA. There was no relationship between the presence of antibodies detected by ELISA and those detected by immunofluorescence, but of 14 IDDM patients with thyroid antibodies, 12 had autonomic nervous system antibodies detected by either immunofluorescence or ELISA (p < 0.005 compared to patients without thyroid antibodies). To further characterize the autoantigen(s), immunoblotting was performed. An adrenal antigen corresponding to 74 kDa was detected in sera from three patients, only one of whom had antibodies detectable by ELISA and immunofluorescence. One IDDM serum showed specific binding to a vagus nerve antigen corresponding to 33 kDa. No specific binding to sympathetic ganglia antigen was demonstrated. Antibodies against autonomic nervous system antigens are an inconsistent feature of diabetes, and appear more associated with coincidental autoimmunity against other organs such as the thyroid.