Neuropathological Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases)

Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed for the following disease entities: CJD - sporadic, iatrogenic (recognised risk) or familial (same disease in 1st degree relative): spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical grey matter; or encephalopathy with prion protein (PrP) immuno-reactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types). Gerstmann-Sträussler-Scheinker disease (GSS) (in family with dominantly inherited progressive ataxia and/or dementia): encephalo(myelo)pathy with multicentric PrP plaques. Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the Fore population). Without PrP data, the crucial feature is the spongiform change accompanied by neuronal loss and gliosis. This spongiform change is characterised by diffuse or focally clustered small round or oval vacuoles in the neuropil of the deep cortical layers, cerebellar cortex or subcortical grey matter, which might become confluent. Spongiform change should not be confused with non-specific spon-giosis. This includes status spongiosus (“spongiform state”), comprising irregular cavities in gliotic neuropil following extensive neuronal loss (including also lesions of “burnt-out” CJD), “spongy” changes in brain oedema and metabolic encephalopathies, and artefacts such as superficial cortical, perineuronal, or perivascular vacuolation; focal changes indistinguishable from spongiform change may occur in some cases of Alzheimer's and diffuse Lewy body diseases. Very rare cases might not be diagnosed by these criteria. Then confirmation must be sought by additional techniques such as PrP immunoblotting, preparations for electron microscopic examination of scrapie associated fibrils (SAF), molecular biologic studies, or experimental transmission.     

Effect of temporal and spatial temperature gradients on the ampullae of Lorenzini

Summary Afferent single fiber impulses were recorded from isolated ampullae of Lorenzini of dogfishes (Scyliorhinus canicula). The ampullae were placed between two thermodes, each of which could be circulated separately with water at 12°, 18° and 24° C, thus allowing cooling and warming with various combinations of spatial temperature gradients. At constant temperature, there was a static discharge in the ampullary fibers. Cooling elicited a dynamic overshoot in frequency, followed by adaptation to a new steady state, whereas warming led to a transient inhibition. Within the limits of error the direction and slope of the spatial temperature gradient had no influence whatsoever on the static and dynamic responses of the ampullae, the only effective parameters being the temperature at the site of the receptor and the rate of temperature change with time.About 13% of the single fibers responded with bursts of impulses, interrupted by silent intervals, and 10% showed an inversed dynamic response, i.e. dynamic overshoot on warming and transient inhibition on cooling. It is possible that these patterns of activity are anomalous responses.This work was supported by the Deutsche Forschungsgemeinschaft.     

Shashi XLMR syndrome: report of a second family

This report describes a family with mental retardation in two brothers. The pedigree is consistent with either X-linked mental retardation or autosomal recessive inheritance. The clinical features consist of coarse face, prominent lower lip, large testes, and obesity. This same constellation of findings was observed in a family with X-linked mental retardation (XLMR) reported by Shashi et al. [2000: Am J Hum Genet 66:469-479]. Furthermore, haplotype analysis was consistent with localization of the Shashi XLMR syndrome in Xq26-q27. Thus, the family likely represents a second occurrence of the Shashi XLMR syndrome.     

Bakteriologische,Serologische und Sero-Cytologische Untersuchungen am Durstenden und Fastenden Menschen

Zusammenfassung Es wird über bakteriologische, serologische und serocytologische Untersuchungen am durstenden und fastenden Menschen berichtet.Bei derSchrothschen Trockensemmelkur kam es zu einer Verschiebung der Darmflora nach der Gram-negativen Seite, trotzdem die gereichte Kost kohlehydratreich ist.Bei derFastenkur blieb die Flora mikroskopisch und kulturell gleich. Erst bei regelwidrigem Fastenbrechen mit großen Mengen Eiweiß wurden die sonst immer in den Aussaaten feststellbaren Kolonien von Bact. coli imperfectum durch typische, Milchzucker vergärende Colikeime verdrängt.Die bakteriologische Untersuchung der Rachenflora bei der Schrothschen Kur ergab ein Verschwinden der in der Vor- und Nachperiode sowie einem Teil des Hauptversuchs nachweisbaren Gram-negativen Catarrhalisflora und das Auftreten von vergrünenden Streptokokken.Die serocytologischen Untersuchungen zeigten, daß sowohl der Opsoningehalt wie die Freßtätigkeit der Phagocyten, unabhängig von den Opsoninschwankungen, durch die Durstkur anders als durch das Fasten beeinflußt werden. Die vonMorgulis vertretene Ansicht, daß die Schrothsche Trockensemmelkur auch nur eine Art Fastenkur ist, und durch Fasten dieselben Erfolge erzielt werden können, ist dadurch hinfällig.Die Untersuchungen über den Komplementgehalt des Serums sowie die Heterolysine gegen Hammelblut, die Isoagglutinine Anti A und B und die Heteroagglutinine gegen Kaninchenblut geben ein Bild von der außerordentlichen Konstanz, mit der diese Antikörper, auch unter schwierigen Verhältnissen, erhalten werden. Dieser Befund bei der Schrothschen Kur entspricht den vonFischer bei derselben Versuchsperson anläßlich einer Fastenkur erzielten Ergebnissen.     

Selective effects of beta-endorphin infused into the hypothalamus, preoptic area and bed nucleus of the stria terminalis on the sexual and ingestive behaviour of male rats

beta-Endorphin was infused bilaterally into the medial preoptic area-anterior hypothalamic continuum at doses of 5, 10 and 40 pmol each side. The highest dose selectively abolished mounting, intromitting and ejaculating in sexually experienced male rats paired with an oestrous female. Males infused with 40 pmol beta-endorphin still followed the female, investigated her anogenital region and other parts of her body, but made abortive attempts to mount. A dose of 5 pmol beta-endorphin had no effect, but 10 pmol proved partially effective. The same males, in other tests, were allowed to ingest a highly preferred, sweet, non-calorific solution (acesulfame-K) in the absence of a female. beta-Endorphin infusions (up to 40 pmol) into the same area of the hypothalamus had no effect on this behaviour. Control males allowed simultaneous access both to an oestrous female and to the sweet solution copulated normally but reduced their ingestive behaviour, despite there being sufficient time during tests for both to occur. beta-Endorphin (40 pmol) infused into the preoptic area-anterior hypothalamic continuum under these conditions suppressed sexual interaction, but ingestion of acesulfame-K increased to values observed when the female was absent. beta-Endorphin infused into neighbouring areas of the brain had different behavioural effects. Sexual behaviour was not inhibited, and ingestion of acesulfame-K was unaltered, when beta-endorphin was infused either into the bed nucleus of the stria terminalis or the rostral ventromedial hypothalamus. However, infusions of cholecystokinin-8 into the ventromedial hypothalamus suppressed acesulfame-K ingestion in most animals, showing that the cannulae were placed in an area regulating ingestive behaviour. The inhibition of sexual behaviour after preoptic area-anterior hypothalamic continuum infusions of beta-endorphin was prevented by either pretreating rats with 1 mg/kg naloxone intraperitoneally, or by infusing a putative delta opiate receptor blocker (0.5 pmols ICI 174864) into the preoptic area-anterior hypothalamic continuum 5 min prior to beta-endorphin treatment. ICI 174864 administered alone significantly increased mount rate and reduced the post-ejaculatory refractory period in copulating males. These experiments suggest that there is both neurochemical and neuroanatomical specificity relating beta-endorphin to sexual behaviour in the male rat.     

ASO Visual Abstract: Will It Play in Peoria? A Pilot Study of a Robotic Skills Curriculum for Surgical Oncology Fellows

Annals of Surgical Oncology -     

State planning for community mental health programs: Implications for psychologists

Official planning bodies in every state are engaged in an intensive two-year analysis of public and voluntary mental health programs. This planning is an integral part of the national effort to provide community mental health services to regions of 75,000 to 200,000 persons. Experiences in establishing and operating the Massachusetts Planning Project are described. Particular attention is paid to the manner in which broad citizen and professional participation has been obtained. Implications for the future functioning of mental health professionals are highlighted. Specific reference is made to the profound crises confronting psychologists and their professional organizations.A brief version of this paper was read at the September, 1964 meeting of the American Psychological Association in Los Angeles.