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  • 1
    ISSN: 1432-1459
    Keywords: Key words Creutzfeldt-Jakob disease ; Alzheimer's disease ; Prion ; Presenilin ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient who was clinically diagnosed with familial early-onset Alzheimer disease (AD) carrying both the E318G substitution in presenilin 1 (PSEN1) and an insertion of 7 octapeptide coding repeats in the prion protein gene (PRNP). Neuropathological examination revealed elongated cerebellar prion protein deposits in the absence of AD pathology. Further analysis of other family members showed that the Creutzfeldt-Jakob disease phenotype in this family was caused solely by the PRNP insertion. This observation is consistent with our previous finding that PSEN1 E318G is not causally related to AD.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1619-7089
    Keywords: Key words: Alzheimer’s disease ; Diagnosis ; Congo red ; Chrysamine G ; Technetium-99m
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Chrysamine G (CG), an analogue of Congo red, is known to bind in vitro to the β-amyloid protein (Aβ 10-43) and to homogenates of several regions of the brain of Alzheimer’s disease (AD) patients. We synthesised a conjugate of 2-(acetamido)-CG with a bis-S-trityl protected monoamide-monoaminedithiol (MAMA-Tr2) tetraligand, which was efficiently deprotected and labelled with a 75% yield with technetium-99m, to obtain 99mTc-MAMA-CG. In mice, 99mTc-MAMA-CG was cleared mainly by the hepatobiliary system, resulting in a fast blood clearance. Brain uptake of 99mTc-MAMA-CG was low. Co-injection with the blood pool tracer iodine-125 human serum albumin (125I-HSA) demonstrated a brain/blood activity ratio for 99mTc-MAMA-CG that was significantly higher than that for 125I-HSA (t test for dependent samples, P〈0.02), indicating the ability of 99mTc-MAMA-CG to cross the blood-brain barrier. In vitro autoradiography demonstrated pronounced binding of 99mTc-MAMA-CG to β-amyloid deposits in autopsy sections of the parietal and occipital cortex of an AD patient as compared with controls. Adding 10 µM Congo red during incubation displaced the binding of 99mTc-MAMA-CG. Congo red staining and autoradiography identified the same lesions. 99mTc-MAMA-CG seems to bind selectively to β-amyloid deposition in human brain parenchyma and blood vessels in vitro and thus might be a lead compound for further development of a useful tracer agent for the in vivo diagnosis of Alzheimer’s disease.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 229 (1983), S. 61-64 
    ISSN: 1432-1459
    Keywords: Wallenberg's syndrome ; Craniopharyngioma ; Syncope ; Cerebral haemorrhage, traumatic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das Wallenberg-Syndrom stellt den klinischen Ausdruck eines Verschlusses der Arterien, die den dorso-lateralen Anteil der Oblongata versorgen, dar, namentlich der A. cerebellaris posterior inferior. Es wird ein Patient beschrieben, bei welchem die typischen Zeichen des Wallenberg-Syndromes mit entsprechender zystischer Nekrose im Ausbreitungsgebiet der oben erwähnten Kleinhirnarterie korrelierten. Ein Kraniopharyngeom hatte sich entlang der Brücke ausgedehnt und umfaßte die A. cerebellaris posterior inferior und die A. basilaris sowie ihre Äste und war für die oben genannte Erweichung im dorso-lateralen Anteil der Medulla oblongata verantwortlich.
    Notes: Summary Wallenberg's syndrome is the clinical manifestation of the obliteration of arterial blood vessels supplying the dorsolateral part of the medulla oblongata, the posterior inferior cerebellar artery being involved in most cases. A patient is described in whom the typical features of Wallenberg's syndrome correlated with cystic necrosis in the perfusion area of the posterior inferior cerebellar artery. A craniopharyngioma spreading along the pons and involving the posterior inferior cerebellar artery, the basilar artery, and its branches was found to be responsible for the infarction of the dorsolateral medulla oblongata.
    Type of Medium: Electronic Resource
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