急性淋巴细胞白血病患者脑脊液中白血病淋巴细胞内DNA、RNA的定量分析

目的：观察脑脊液白血病淋巴细胞脑鞘注治疗前后细胞内DNA、RNA相对含量的变化。方法：采用激光扫描共聚焦显微术（LSCM）对15例急性淋巴细胞性白血病患者脑脊液中550个白血病淋巴细胞和鞘注治疗后脑脊液中380个白血病淋巴细胞内DNA、RNA相对含量进行定量检测,同时用荧光强度地形分布图像表示,并与正常脑脊液中小淋巴细胞内DNA、RNA相对含量进行对比。结果：脑脊液白血病巴细胞内DNA、RNA相对含量显著高于正常对照（P＜0．01）;鞘注治疗后细胞内DNA、RNA相对含量明显低于治疗前（P＜0．01）。且三者DNA、RNA荧光强度地形分布立体图像也存在着显著的判别;治疗前似圆柱菜,其顶部显强白色荧光,连成一片,完整无损,治疗后呈锯齿状,顶部显白色荧光,较治疗前弱,山峰较多,分散,连连;对照组似圆锥形,顶部显白荧光。结论：利用LSCM对脑脊液白血病淋巴细胞内DNARNA相对含量的定量分析可为CNLL诊断,鉴别诊断以及化疗药物的筛选、疗效评价,预后判断提供可靠的实验室依据。     

检测病毒性脑炎患儿脑脊液中单纯疱疹病毒IgM抗体的临床应用

为探讨单纯疱疹病毒脑炎的早期诊断方法 ,用 EL ISA法检测 10 5例小儿病毒性脑炎脑脊液 (CSF)中特异性单纯疱疹病毒 Ig M抗体 ,为对比同时也检测了该组病毒特异性 Ig G抗体 ,并与血清相应抗体结果比较。其中 13例阳性 ,占 12 .4%。结果表明 :检测 CSF中单纯疱疹病毒 Ig M抗体 ,可以快速确定其疱疹病毒性脑炎 ,方法简单易行 ,具有其临床实用价值     

Overdrainage and shunt technology

When vertical body position is simulated, conventional differential pressure valves show an absolutely unphysiological flow, which is 2–170 times the normal liquor production rate. Although this is compensated in part by the resistance of the silicon tubes, which may produce up to 94% of the resistance of the complete shunt system, a negative intracranial pressure (ICP) of up to 30–44 cmH₂O is an unavoidable consequence, which can be followed by subdural hematomas, slit ventricles, and other well-known complications. Modern shunt technology offers programmable, hydrostatic, and flow-controlled valves and anti-siphon devices; we have tested 13 different designs from 7 manufacturers (56 specimens), using the Heidelberg Valve Test Inventory with 16 subtests. Programmable valves reduce, but cannot exclude, unphysiological flow rates: even in the highest position and in combination with a standard catheter typical programmable Medos-Hakim valves allow a flow of 93–232 ml/h, Sophy SU-8-valves 86–168 ml/h with 30 cmH₂O. The effect of hydrostatic valves (Hakim-Lumbar, Chhabra) can be inactivated by movements of daily life. The weight of the metal balls in most valves was too low for adequate flow reduction. Antisiphon devices are highly dependent on external, i.e. subcutaneous, pressure which has unpredictable influences on shunt function, and clinically is sometimes followed by shunt insufficiency. Two new Orbis-Sigma valves showed relatively physiological flow rates even when the vertical position (30 cmH₂O) was simulated. One showed an insufficient flow (5.7 ml/h), and one was primarily obstructed. These have by far the smallest outlet of all valves. Additionally, the ruby pin tends to stick. Therefore, a high susceptibility to obliterations and blockade is unavoidable. Encouraging results obtained in pediatric patients contrast with disappointing experiences in some German and Swedish hospitals, which suggests that our laboratory findings are confirmed by clinical results. The concept of strict flow limitation seems to be inadaequate for adult patients, who need a relatively high flow during (nocturnal) ICP crises. The problem of shunt overdrainage remains unsolved.This paper is an updated version of an oral presentation held at the Consensus Conference: Hydrocephalus '92 Assisi, Italy, 26–30 April 1992Deceased     

云南哈尼族CSF1PO,TPOX和THO1基因座多态性分析

应用３个ＳＴＲ基因座在同一反应体系中进行互不干扰的多重ＰＣＲ,采用高分辨力的聚丙烯酰胺弟胶电泳分离、银染法显影技术,对云南哈尼族的ＣＳＦ１ＰＯ,ＴＰＯＸ和ＴＨＯ１等３个基因座等基因的基因频率进行调查,ＣＳＦＩＰＯ基因座,观察到７个等基因,１７个基因型;ＴＰＯＸ基因座,以６个等基因,１３个基因型;ＴＨＯ１基因座,观察到６个等基因,１９个基因型,获得了满意的结果,显示了广阔的应用前景。     

云南拉祜族六个基因座的遗传多态性研究

目的：了解云南拉祜族６ 个短串联重复序列基因座遗传多态性分布。方法：应用复合扩增技术对ＣＳＦ１ＰＯ、ＴＰＯＸ、ＴＨ０１ 、Ｆ１３Ａ０１、ＦＥＳＦＰＳ和ｖＷＡ等６ 个ＳＴＲ基因座进行分析,采用高分辨率的聚丙烯酰胺凝胶电泳分离、银染法显影技术,对云南拉祜族上述６ 个ＳＴＲ基因座进行遗传多态性调查。结果：ＣＳＦ１ＰＯ基因座,观察到８ 个等位基因,２０ 个基因型;ＴＰＯＸ 基因座,观察到５ 个等位基因,１４ 个基因型;ＴＨ０１ 基因座,观察到６ 个等位基因,１７ 个基因型;Ｆ１３Ａ０１ 基因座,观察到６ 个等位基因,１３ 个基因型;ＦＥＳＦＰＳ基因座,观察到７ 个等位基因,１５ 个基因型;ｖＷＡ基因座,观察到７ 个等位基因,１７ 个基因型。结论：上述６ 个短串联重复序列基因座基因频率分布与Ｈａｒｄｙ Ｗｅｉｎｂｅｒｇ 平衡吻合良好。     

Shunt complications in the first postoperative year in children with meningomyelocele

The authors analyze the incidence of early mechanical and infective CSF shunt complications and various factors that might be correlated with the incidence in a series of 170 children affected by hydrocephalus and meningomyelocele (MM), with the aim of finding specific risk factors related to this particular type of hydrocephalus. Factors investigated for correlation with CSF shunt malfunction are the following: level of the spinal malformation, age of the patient at MM repair, age at diagnosis of hydrocephalus, degree of the ventricular dilatation, age at CSF shunt implantation, modality of the surgical procedure, characteristics of CSF at operation. In the first postoperative year following CSF shunting, 45.9% of the patients presented one shunt malfunction, three-quarters of which were due to mechanical causes, and one quarter to infection. Age of the patient at diagnosis of hydrocephalus and at CSF shunt operation did not significantly influence shunt patency, nor did the surgical modality (programmed vs emergency procedure). On the other hand, MM level did influence the outcome of CSF shunting: a higher percentage of malfunctions (and in particular of infective complications) was observed among the patients with high level MMs than in the group with more caudal location of the spinal defect. Similarly, the degree of ventricular dilatation correlated with the incidence of complications (more severe ventricular dilatation was associated with the highest incidence of complications). The order in which MM repair and CSF shunting were carried out and the age of the patients at MM repair did not affect the occurrence of mechanical complications, whereas they had a significant effect on the incidence of infective complications. In fact, the rate of overall complications, and of infective complications in particular, was proportional the age at MM repair. Furthermore, the group of children who underwent to MM repair and CSF shunting simultaneously scored the lowest percentage of complications, although these were mainly infections; the highest incidence of complications (and in particular of infective ones) was observed in the children who underwent CSF shunting first. The most striking correlation, however, was found with the characteristics of CSF. While normal CSF values correlated with an overall incidence of complications of 39.2%, abnormal CSF values were correlated with a rate of complications of 90.9%; in particular, the rates of infective complications were 2.7% and 77.3%, respectively. On the grounds of these observations a protocol is proposed of temporary CSF external drainage in children requiring prompt relief of increased intracranial pressure but at risk for the presence of a leaking spinal defect or of a MM left unrepaired for more than 48 h.     

The effect of l-dopa and propranolol on human CSF cyclic nucleotides

Human CSF cyclic AMP and cyclic GMP have been measured as possible indicators of activity of central neurotransmitter-sensitive adenylate or guanylate cyclase. In an attempt to help to identify the specific neurotransmitter systems of origin of human CSF cyclic AMP and GMP, we studied Parkinson patients with and without l-dopa therapy and schizophrenic patients before and after propranolol therapy. No effect of l-dopa or propranolol was found on CSF cyclic nucleotides. However, Parkinson patients had a 40–50% reduction of CSF cyclic AMP and a 80–90% reduction of CSF cyclic GMP compared with the schizophrenic patients. Implications of this finding are discussed.     

Cerebral disseminated coagulation

Summary The distribution of cytarabine in the ventricular CSF was studied after lumbar intrathecal administration. In the ventricular fluid peak concentrations up to 1.5×10^–4 M (37 g/ml) were achieved 4–8 h after lumbar injection of cytarabine of 45 mg/m². The concentration of cytarabine decreased slowly with a half-time of 2.16 h thus providing therapeutic concentrations in the cerebral ventricles for more than 24 h.

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Zusammenfassung Die Verteilung von Cytosinarabinosid nach lumbaler intrathekaler Injektion im ventrikulären Liquor cerebrospinalis wurde untersucht.Nach lumbaler intrathekaler Injektion von 45 mg Cytosinarabinosid (Alexan®) pro m² Körperoberfläche wurde im ventrikulären Liquor eine Höchstkonzentration von 1,5×10^–4 M (37 g/ml) 4–8 h nach Applikation erreicht. Die Konzentration von Cytosinarabinosid im ventrikulären Liquor fiel langsam mit einer Halbwertszeit von 2,16 h ab. Bei lumbaler Applikation wurden bei dieser Untersuchung therapeutische Konzentrationen von Cytosinarabinosid in den cerebralen Ventrikeln für mehr als 24 h erreicht.

     

Postural imbalance: An early sign in HIV-1 infected patients

Summary We have recorded postural performance in 50 HIV-infected patients in different stages of the disease (Walter Reed (WR) stages I–VI) by means of a force measuring platform. The results were compared with 50 age-matched controls. A significant instability was particularly evident when standing on an unstable foot support. In patients standing with eyes closed, postural sway was significantly higher in every patient group (WR I–II:P<0.02, WR III–V:P<0.001, WR VI:P<0.001). Patients in stage WR I–II showed no relevant neurological abnormalities. In agreement with other neurophysiological data in the literature we suggest that postural imbalance could be an early sign of central nervous system penetration of HIV. No correlation with electromyographic or cerebrospinal fluid findings could be found.     

Huntington's Disease — Imbalance of free amino acids in the cerebrospinal fluid of patients and offspring at-risk

Summary A total of 27 different amino acids were determined in the fasting, morning lumbar CSF of 12 patients with Huntington's Disease (HD), 8 at-risk offspring and 16 non-choreic control patients. A significant (P<0.001) decrease was observed for asparagine, isoleucine, leucine, phenylalanine, histidine, arginine, -aminoadipic acid and homocarnosine in patients with HD compared to the non-choreic controls. Only tyrosine was increased in HD. These alterations were to an extent more pronounced in 5 neurophysiologically conspicuous offspring. The alterations suggest that amino acid imbalance is an early metabolic disturbance in HD.

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Zusammenfassung Bei 12 Patienten mit manifester Huntingtonscher Krankheit (HD), 8 nicht erkrankten Nachkommen und 16 nicht choreatischen Kontrollpatienten wurden 27 verschiedene Aminosäuren im Liquor cerebrospinalis (nüchterner Lumballiquor) untersucht. Asparagin, Isoleucin, Leucin, Phenylalanin, Histidin, Arginin, -Aminoadipinsäure und Homocarnosin waren signifikant (P<0.001) erniedrigt bei Patienten gegenüber den Kontrollen. Diese Veränderungen waren bei 5 neurophysiologisch auffälligen Nachkommen teilweise ausgeprägter, was für eine frühzeitige metabolische Störung spricht. Tyrosin war als einzige Aminosäure erhöht. Die Veränderungen sprechen für eine Aminosäurenimbalanz bei Huntingtonscher Krankheit, deren Bedeutung besprochen wird.