Friedreich's ataxia presenting with pure sensory ataxia: a long-term follow-up study of two patients

We describe two patients with Friedreich's ataxia whose presenting symptomatology was for years progressive tabetic ataxia. Based upon the initial clinical, electrophysiological and nerve biopsy data, a diagnosis of idiopathic sensory neuropathy was established. Subsequent examination of the kin showed that three sisters of case 1 had Friedreich's ataxia. Upon serial clinical and electrocardiographic study, both patients eventually developed a florid Friedreich's ataxia, including cardiomyopathy. Our findings indicate that at onset Friedreich's ataxia may be indistinguishable from sensory neuropathy and also that serial examination and investigation of kinship are essential steps for accurate diagnosis.     

Multidetector single-photon emission tomography: are two (or three or four) heads really better than one?

Today, almost every camera sold, with the exception of mobile systems, is single-photon emission tomography-capable. The atest technical development is the so-called multidetector systems. This article reviews the development and current and potential clinical role of these multidetector systems, with particular emphasis on multicamera systems. The utility of multidetector systems is considered in the context of an image's signal-to-noise ratio, including its effect on diagnostic or quantitative accuracy. The potential for multidetector systems to increase the signal-to-noise ratio is discussed, as are other potential benefits. Finally, advice on an approach to equipment selection is provided.     

Hemispherectomy — 25 years later — findings and concepts

Summary In the first part the case of a then 27 year old female patient with right-sided infantile spastic hemiplegia after left-sided porencephaly is described, in whom hemispherectomy was performed 25 years ago. The postoperative state and the development are described as well as the social outcome and the present neurological status. A computer-tomogram shows the actual state of the brain.The second part is devoted to a scientific discussion of the supplementary motility after pyramidal lesions which is defined as the action of descending subcortico-spinal pathways starting probably in the mesencephalon, whereas an ipsilateral pathway is unlikely. Comparative neurological experiments serve to support such a concept as well as similar observations in traumatic cerebral lesions in man. In the light of the far more skilled motility in cerebral lesions of the young as well as the possibility of a shift of neuropsychological functions of the dominant to the contralateral hemisphere in children up to the age of 8–10 the possibility is discussed that plasticity — the concept of Albert Bethe — could form the mechanism of auxiliary function.Furthermore the surprising sensory performances in some of the patients with hemispherectomy are emphasized and the possibilities of anipsilateral substratum are discussed; however, this contrasts with the concepts formulated for the auxiliary motility after pyramidal lesions. Clarification of these problems will probably follow only after further experimental work.

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Zusammenfassung Im ersten Teil der Arbeit wird der Fall einer damals 27-jährigen Patientin beschrieben, die eine rechtsseitige infantile spastische Hemiplegie nach linksseitiger Porencephalie hatte. Bei ihr war 25 Jahre zuvor eine Hemisphärektomie durchgeführt worden. Der postoperative klinische Status und die Entwicklung der Syndrome wird beschrieben und über die soziale Situation berichtet. Schließlich wird der derzeitige neurologische Status genau wiedergegeben. Erstmalig zeigt ein Computer-Tomogramm den Zustand des Hirns nach diesem Zeitraum.Im zweiten Teil der Arbeit wird die Entstehung der Ersatz-Motilität diskutiert, die als eine Aktion deszendierender subkortiko-spinaler Bahnen definiert wird, die wahrscheinlich vom Mittelhirn ihren Ausgang nehmen, während die Tätigkeit ipsilateraler Systeme für unwahrscheinlich gehalten wird. Vergleichende Tierexperimente am Hirn unterstützen eine solche Auffassung, ebenso wie auch die Beobachtung bei umschriebenen Hirnverletzungen des Menschen.Dann wird die Möglichkeit von Ersatzfunktionen durch Plastizität diskutiert — ein Begriff von Albert Bethe. Dies erscheint im Lichte vieler Beobachtungen wichtig, da die Ersatz-Motilität bei Hirnläsionen junger Menschen sehr viel höhere Geschicklichkeit ermöglicht als bei Läsionen von Älteren; weiter ist zu beachten, daß neuropsychologische Funktionen bei einer Hirnläsion vor dem 8. bis 10. Lebensjahr von einer dominanten Hemisphäre noch auf die andere Seite verlegt und dort lokalisiert werden können. Schließlich wird bei diesen Patienten auf die erstaunlichen Fähigkeiten der sensiblen Systeme nach Hemisphärektomie hingewiesen und hier die Möglichkeit eines ipsilateralen anatomischen Apparates diskutiert. Jedoch können bei der Sensibilität so genaue funktionell-morphologische Vorstellungen noch nicht formuliert werden, wie sie sich z. Zt. schon für die Ersatz-Motilität nach Pyramidenbahnläsion ergeben. Erst genauere Experimente im Tierversuch können weitere Aufklärung bringen.

     

Solubilization and Stabilization of a Benzylpenicillin Chemical Delivery System by 2-Hydroxypropyl-β-cyclodextrin

A dihydropyridine pyridinium salt redox carrier-based chemical delivery system for benzylpenicillin (1) was complexed with 2-hydroxypropyl--cyclodextrin (HPCD). The solubility of the lipophilic 1, which is incompatible with aqueous formulations, was dramatically increased and showed a linear dependency on the HPCD concentration. The degree of incorporation was 20 mg of 1 per g of complex. The stability study of 1 in various pH buffers indicated the base-catalyzed hydrolysis of the acyloxyalkyl linkage and the hydration of the 5,6 double bond of the dihydropyridine as the main degradation processes. The overall loss of 1, which follows first-order kinetics, was not influenced by changes in ionic strength and elimination of oxygen from the reaction medium. The HPCD complex of 1, which has a stability constant of 720–940 M ^–1, stabilized the chemical delivery system. The influence of the temperature on the stability of 1 is also discussed.     

支气管扩张症中神经内分泌免疫网络及肥大细胞的变化

目的：探讨支气管扩张症中神经内分泌免疫网络的异常和肥大细胞（MC）在该病发病中的作用及关系。方法：应用组织化学、免疫组化、组织化学与免疫组化结合的方法和形态计量学方法进行观测。结果：支气管扩张症中，支气管上皮蛙皮素（Bombesin）阳性细胞、固有膜S-100蛋白和神经特异性烯醇化酶（NSE）阳性神经纤维、IgE阳性细胞、MC和IgE阳性MC均显著增多，且在支气管相关淋巴组织（BALT）增生的区域上述肺内分泌细胞、神经纤维和IgE阳性细胞增多尤为显著，S-100蛋白和NSE阳性神经纤维分布于弥散淋巴组织和BALT中，MC与S-100蛋白阳性神经纤维紧密接触．MC表面有IgE阳性环状带，MC和IgE阳性细胞出现在支气管上皮间和肺泡壁。结论：支气管扩张症的发病与局部神经内分泌免疫网络异常有关；MC可能作为感受器、分泌细胞或靶细胞参与神经内分泌免疫网络，在支气管扩张症的发病中起重要作用。     

Lateral asymmetries in the trigeminal ganglion of the male rat

We have applied stereological methods to estimate the number and perikaryal size of primary sensory neurons in celloidin-embedded trigeminal ganglia of male albino rats, specifically looking for inter-individual and side variability. The mean total number of neurons per ganglion was 35,300, with a moderate variability among ganglia. On average, 66% of the neurons were classified as A-type and 34% as B-type. Although for individual cases there could be notable side differences in the number of neurons of each type, on a population basis these differences were not significant. Mean neuronal volume was four times larger for A- than for B-cells, and both populations exhibited a moderate variability among individuals. High intra-animal side differences were found for A-cells, which were on average a significant 23.5% larger in the right ganglia. B-cells did not show significant side differences. The distribution of individual volumes around the mean value was consistently skewed to the right, particularly in the case of A-cells, which partially overlapped with the largest B-cells. In the right ganglion the distribution of A-cells, but not of B-cells, showed a rightward bias, revealing the increase in bigger neurons. The existence of larger A-type neurons in the right trigeminal ganglion may provide a structural substrate for some somesthetically based complex behaviors which are best performed by rats using their right vibrissae.     

Inflammation-induced changes in primary afferent-evoked release of substance P within trigeminal ganglia in vivo

Substance P (SP) is synthesized in a subset of nociceptive sensory neurons and is released from their peripheral and central terminals. Here we demonstrate with the use of in vivo microdialysis and radioimmunoassay techniques that SP is also released within trigeminal ganglia following intraganglionic application of KCl, veratridine or capsaicin, and after electrical stimulation of peripheral afferent fibers. Both the basal and KCl-evoked release of SP are shown to be dependent on extracellular calcium. Using the turpentine-induced model of unilateral orofacial inflammation we also show that both the basal and KCl-evoked release of SP within trigeminal ganglia are greatly increased on the inflamed side 48 h after induction of inflammation. Coupled with previous demonstrations of excitatory effects of SP on sensory neurons, these results suggest that SP fulfils the role of a non-synaptically released diffusible chemical messenger that may modulate the somatic excitability of neurons within sensory ganglia in inflammatory pain states.     

青光眼的中枢损伤

青光眼性损伤的形态学特征及病理机制 ,在视网膜节细胞水平及视神经水平已得到广泛研究 ;最近研究表明 ,青光眼对外侧膝状体、视皮质等中枢视路也存在相应损伤 ,并且提示青光眼的中枢损伤可能参与视功能状态的进一步恶化。我们对近年来青光眼中枢损伤的病理形态学及病理机制研究作一综述。     

Histopathologic parameters of prognosis in cervical cancer – a review

Apart from clinical stage and lymph node status, acknowledged to be among the most powerful predictors of outcome in cervical cancer, the determination of prognosis and thereby the need for adjuvant therapy in surgically treated patients currently relies on a variety of histopathologic factors. The role of many of these is controversial. This may be because histopathology is genuinely lacking in sensitivity for predicting tumor behavior in vivo. There is, however, wide variation in histopathologic definitions and criteria. This is probably the major reason for both the lack of reproducibility in the reporting of certain factors and in their diminished value in predicting behavior. Tumor type, grade, vascular invasion, pattern of invasion, and depth are all extremely important prognostic indicators when used individually or as a part of a scoring system.     

中国科技期刊如何进入国际信息检索系统

以被多种国际著名信息检索系统收录的World Journal of Gastroenterology（WJG）杂志的发展历程为样例。探讨如何提高中国出版的科技期刊被国际信息系统收录的命中率，提高期刊的国际地位和知名度，更好地促进与国际的学术交流。