ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01297.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

The subjects were 98 children with an average age of 5.8 years. The frequent seizures were categorized into the following three modes: (1) seizures occur more than five times a day; (2) 1 ‐4 seizures occur every day for one week or more; (3) 1 ‐4 seizures a day occur weekly for one month or more. The incidence of each mode was as follows: (1) 15.3%; (2) 16.3% (3) 173% (1) + (2) 43.9% etc. We observed frequently the cases of tonicclonic seizures in mode (l), infantile spasms (IS) in mode (2), simple partial seizures (SPS) in mode (3), and minor seizures, IS, and Lennox‐Gastaut syndrome (LCS) in mode (1) + (2). Abnormal CT findings were observed in 37.8%. Of all the patients, 33.7% indicated a developmental quotient (DQ) or intelligence quotient (IQ) above 80, and 31.6% showed a DQ or IQ under 30. Of 96 patients followed for the past one year or more, 32.3%, consisting mainly of the cases with absence seizures and IS, had been seizure‐free for at least one year; 60.4% including the cases with LGS, SPS, etc., still had s

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01298.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Current knowledge suggests integration of cerebral perfusion and metabolism as enabling normal neuronal function, and their pertubations explaining the brain damage of hypoxia, hypoglycaemia, hypoperfusion and status epilepticus. Similar mechanisms appear operative in the viral encephalopathies and cause psychomotor dysfunction and epilepsy. A transient inhibition of plasma membrane glucose transport is central to the understanding of the metabolic abnormalities of these encephalopathies, the ensuing cell energy crisis resulting from neuroglycopoenia being evidenced by electroencephalographic changes, lactic and ketoacidosis, hyperuricaemia and ionic aberrations. Failure of Na+ and Ca2+pumps cause cerebral oedema and neuronal death respectively, the selective nature of the latter being due to α‐adrenergic vasoconstriction. Management with hyperglycaemia‐producing infusions and the judicious use of lactate and steroids can overcome the transport dysfunction and enable complete recovery. The temporal profile of the metabolic aberrations of febrile convulsions, which are the result of adaptation, provide a template supporting this mode of management of the severe encephalopat

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01299.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

AbstractRemarkable progress has been accomplished in epileptology in recent years. The remission rate in childhood epilepsy has been improved by the introduction of new technologies and better diagnostic as well as therapeutic methods. Investigation of prognosis is an important field of epileptology, because prognostic improvement reflects its overall progress, A long‐term follow‐up study of childhood epilepsy was undertaken in the Okayama University Hospital. Ten to 15 years of follow‐up was possible in 730 of 1,295 patients who were first diagnosed at ages below 15 years, from 1968 to 1971. The 3‐year remission rate amounted to 82.0% and 5‐year remission was obtained in 79.1%. These high rates of remission indicate the favorable prognosis of childhood epilepsy. On the other hand, cases of intractable epilepsy also amounted to a considerable number. Intractable epilepsy consisted mainly of agedependent epileptic encephalopathy (Ohtahara's syndrome, West syndrome and Len‐nox‐Gastaut syndrome) and severe myoclonic epilepsy in infancy. Development of effective therapy for these intractable epileptic syndromes will be an important subject of f

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01300.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01301.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

It is controversial whether developmental retardation occurs in children with epilepsy and what causes the retardation if it occurs. The present study aims to reveal the factors associated with the retardation. The patients were 126 children who had been developmentally normal before the onset of epilepsy and had been followed for more than five years. Fortyeight of the 126 children showed mental retardation, and the remaining 78 had normal mental development at the follow‐up. Developmental retardation was observed in children with onset before one year, known etiology, diffuse slow spike‐wave or hypsarrhy‐thmia on EEC, ingestion of six or more drugs and high blood levels of phenobarbital or phenytoin. Late onset of epilepsy, idiopathic or familial etiology, ingestion of five drugs or less and low blood level of phenobarbital or phenytoin were associated with normal develo

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01302.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Epilepsy and epileptic syndromes are one of the major pediatric neurological diseases in Taiwan, R.O.C. In 1984 we investigated 38 elementary schools in Taichung city, in the middle‐west part of Taiwan. Among 57,944 school‐aged children, 388 had suffered from at least two episodes of afebrile seizures occurring separately over a two‐week period. The period prevalence rate was 0.67%. In our clinic, once seizures are diagnosed, the seizure types are then classified as well as possible, in order to develop a rational approach to treatment. We commonly use carbamazepine for partial seizures and some generalized seizures, low dose ACTH (10 I.U.) for infantile spasms, valproic acid for absence seizures and myoclonic seizures, clonazepam for atonic‐akinetic and myoclonic seizures, and phenobar‐bital for young children with generalized seizures. In the last three years, we have used a ketogenic diet for akinetic‐atonic seizures and Lennox‐Gastaut syndrome; we have also used temporal lokctomy for complex partial seizures since 1981. Owing to the marked increase in the medical understanding of epileptic seizures and syndromes, and models of treatment, there is no question that children with seizure disorders are better off today than they were

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01303.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

The leukotrienes C4, D4and E4are potent biologic mediators, and are thought to play an important role in obstructive airways disease such as asthma. In this study, plasma im‐munoreactive LTC4(iLTC4) levels in asthmatic children were measured using radio‐immunoassay after Sep‐pak extraction in order to determine whether LTC4is releasedin vivoduring an asthmatic attack. In 10 non‐atopic children, the mean ± SEM of plasma iLTC4level was 0.031 ± 0.013 pmol/ml. Significantly higher plasma iLTC4levels were recognized at all stages in asthmatic children, both in remission (p<0.01) and during an attack (p<0.01). PaO2levels during an attack were significantly lower in the high iLTC4group. In 15 asthmatic children, the plasma iLTC4levels during an attack (0.134 ± 0.017 pmol=ml) significantly decreased during recovery (0.078 ± 0.012 pmol/ml) (p<0.05). Plasma iLTC4levels correlated closely with lung function (p<0.01). The high iLTC4levels in plasma in asthmatic children suggest a role for LTC4in the pathophysiolog

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01304.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Peptide leukotrienes C4, D4and E4are considered to be major mediators of hypersensitivity reactions. In this study, immunoreactive leukotriene C4(iLTC4) in plasma, taken from asthmatic children who had undergone bronchial challenge with a specific allergen, was measured using radioimmunoassay in order to determine whether LTC4is releasedin duoduring human allergic reactions. With regard to the bronchial challenge of 10 asthmatic children, plasma iLTC4levels at the postchallenge stage (0.131 ± 0.037 pmol/ml) were significantly elevated compared to the prechallenge stage (0.065 ± 0.016 pmol/ml) (p<0.01). The bronchial response was not directly related to the increased plasma iLTC4levels, but the strength of the bronchial response did relate to the plasma iLTC4levels at the pre‐challenge (p<0.05) and postchallenge (p<0.01) states. The prechallenge levels are relevant to bronchial reactivity, and the post‐challenge levels are relevant to bronchocon‐striction caused by exposure to allergens. Thein vivorelease of LTC4following exposure to a specific allergen suggests that leukotrienes are important in the pathogenesis of

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01305.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

The isoenzyme of hypoxanthine‐guanine phosphoribosyltransferase (HPRT, E.C.2.4.2.8) functions in the metabolic salvage of purines. Partial HPRT deficiency is associated with gouty arthritis, while absence of activity results in Lesch‐Nyhan (LN) syndrome. We characterized five unrelated patients with HPRT deficiency to understand the spectrum of molecular defects using Southern and Northern blot, polymerase chain amplification of HPRT mRNA and DNA sequencing, and oligonucleotide hybridization analysis of the HPRT gene. Southern blot analysis of DNA indicated that mutations leading to HPRT deficiency in our five patients were not the result of major chromosomal rearrangements or deletions. Sequencing analysis of the amplified DNA from three different patients with HPRT deficiency implied three unique molecular abnormalities: 1) one single‐base substitution at codon 54 (from ATG to CTG) resulting in the replacement of methionine with leucine in an LN patient, 2) two single‐base substitutions at codon 179 (from GTT to GGT) and at codon 180 (from GGA to AGA) resulting in the replacement of valine with glycine and glycine with arginine in a gouty patient, and 3) 51 nucleotide deletion between nucleotides 747 and 797 resulting in the formation of shorter sized HPRT mRNA and putative two amino‐acid deleted HPRT protein in another gouty patient. These results are the direct molecular evidence of genetic heterogeneity in mu

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1989.tb01306.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY