Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease

The objective of the present study was to compare quantitatively the neuropathology of two subtypes of Creutzfeldt-Jakob disease (CJD), viz., sporadic CJD (sCJD) and variant CJD (vCJD). The vacuolation (‘spongiform change’), surviving neurons, glial cell nuclei, and deposits of the disease form of prion protein (PrPsc) were quantified in histological sections of the cerebral cortex, hippocampus, and cerebellum in 11 cases of sCJD and 15 cases of vCJD. Three aspects of the quantitative pathology of each histological feature were studied: overall abundance (density or coverage), spatial distribution parallel to the tissue boundary, and laminar distribution across gyri of the cerebral cortex. Overall vacuole density was greater in sCJD than in vCJD in some regions while overall neuronal densities were greater in vCJD. In cerebral cortex, vacuoles and PrPsc deposits were distributed in clusters which exhibited a regular distribution parallel to the pia mater, this type of spatial pattern being more frequent in sCJD than in vCJD. In some cortical gyri there were differences in laminar distribution between subtypes, viz. the vacuolation was more generally distributed across cortical laminae in sCJD, neuronal loss was often greater in upper laminae in vCJD but in lower laminae in sCJD, and PrPsc deposits were more frequently distributed in upper laminae in vCJD but in lower laminae in sCJD. A significant gliosis affected lower cortical laminae in both sCJD and vCJD. Hence, there were differences in degeneration of cerebral cortex, hippocampus, and cerebellum in sCJD and vCJD, which may reflect variations in disease aetiology and propagation of PrPsc through the brain.

Effect of reading sport chronicles on the perception of justice and the predisposition to protest actions in soccer

In this study three chronicles from national newspapers (one generalist and two sport press) were analyzed. The chronicles belong to Spain’s soccer final of the King’s Cup in 2014. The aim of the study was to know if there was any influence on the readers’ perception of justice and consequently if this influence could cause a particular predisposition to participate in acts of protest. 462 university students participated. The results showed that different chronicles caused differences in the perception of justice depending on the chronicle read. However, a clear influence on the willingness to participate in acts of protest was not obtained. These results should make us think about the impact of sport press and its influence, and to be aware of the indirect responsibility of every sector on the antisocial behaviors generated by soccer in our country.

Sorge-qwäde öfver doctorinnan fru Elisabeth Stråhlmans född Tavast, dödliga frånfälle, stäldt till deltagande slägtingar och wänner vid hennes begrafning å Walkjärvi prestegård, den 29 januarii 1834 af dess bedröfvade make Johan Stråhlman

Ruotsinkielinen runo.

Johan Bunianin elämäkerta

Nimiösivulla myös: Saksasta suomeksi käännetty. - Nimiösivulla myös motto: Kussa synti on suureksi tuttu, siinä on armo ylönpaltiseksi tuttu. (Rom. 5,20).

Pieni Kempi, eli, Lyhykäiset opetukset ja rukoukset pienille ja yksinkertaisille kokoon kouttu Thomas Kempin pienistä kirjoista. Lisäksi otettu wiisi sangen merkillistä lukua Johan Arndin Totisesta christillisyydestä

Pippingin mukaan suomentanut Henr. Renqvist. - Laineen mukaan kustantaja todennäköisesti Henrik Renqvist.

Jakob Benecke (Hrsg.): Die Hitler-Jugend 1933 bis 1945, Programmatik, Alltag, Erinnerungen. Eine Dokumentation, Weinheim / Basel: Beltz Juventa 2013 [Rezension]

Rezension von: Jakob Benecke (Hrsg.): Die Hitler-Jugend 1933 bis 1945, Programmatik, Alltag, Erinnerungen. Eine Dokumentation, Weinheim / Basel: Beltz Juventa 2013 (418 S.; ISBN 978-3-7799-2651-1; 39,95 EUR)

Altered regulatory response of Rab7a and Rab9 in MM1 and VV2 subtype of Creutzfeldt-Jakob disease

The present study was undertaken to identify proteins interacting with PrPC that could provide new insights into its physiological functions and pathological role. We performed a target search for lysosomal network protein, Rab7a and Rab9, in frontal cortex and cerebellum of human brain from patients with sCJD-MM1 and sCJD-VV2. The intracellular level of Rab7a was increased significantly, when compared with healthy age-matched control. Interactions of PrPC and Rab7a/Rab9 were further investigated by using confocal laser scanning microscopy. Immunofluorescence results suggested potential interactions of Rab7a and PrPC. siRNA against the Rab7a gene was used to knockdown the expression of Rab7a protein in primary cell culture of cortical neurons from wild type mice. This depleted Rab7a resulted an impairment of PrPC trafficking leading to an accumulation of PrPC in the endocytosis pathway. Furthermore, interactions of Tau and Rab7a were investigated by using western blot analysis and confocal laser scanning microscopy. Cell cultures of cortex of wildtype mice were treated with siRNA-Tau, siRNA-Rab7 and control siRNA followed by immunofluorescence. The results of immunofluorescence suggested potential interaction of Tau and Rab7a. Cells lines treated with siRNA-Tau, the intracellular levels of Rab7a and Rab9 significantly increases and their localization is also modified. When we transfected this cells lines with siRNA-rab7a the accumulation of Tau decreases in cytosolic region and their localization was also modified when compared with control cells. In conclusion, this study may help to understand and characterize the subtype specific disease progression in CJD cases. Furthermore, it could be a step ahead to development of new treatment strategies for diseases subtype specific manner.

”Länsikauppakin meille sentään jotakin merkitsee” – Johan Nykopp Suomen Washingtonin -lähettiläänä 1951–1958.

Tutkielmani aiheena on Johan Nykoppin toiminta Suomen Washingtonin -lähettiläänä vuosina 1951–1958. Tutkimuskirjallisuudessa Nykoppia on kuvailtu länsimieliseksi lähettilääksi, joka pyrki työssään vahvistamaan Suomen läntisiä talousyhteyksiä harvinaisen aktiivisesti. Pelkästään häntä koskevaa tutkimusta ei kuitenkaan aiemmin ole tehty. Työssäni selvitän, miten Nykopp käytännössä edisti tavoitettaan Suomen viennin kasvattamisesta ja sen suuntaamisesta länteen. Lisäksi tarkastelen, miten hän kuvasi raporteissaan Suomen ja Yhdysvaltain suhteita ja millaista politiikkaa hän suositteli niissä kotimaan päättäjille. Lähteinä käytän pääasiassa Nykoppin diplomaattiraportteja sekä kirjeenvaihtoa lähettiläskauden ajalta. Niiden antamaa kuvaa täydennän tutkimus- ja muistelmakirjallisuuden avulla. Osoitan tutkielmassani, että Nykoppilla oli lähettiläskautensa aikana merkittävä panos Suomen ja Yhdysvaltain suhteiden kehittämisessä. Tutkimustulokseni vahvistavat tulkintaa, jonka mukaan Nykoppin toimintaa Suomen ulkomaankaupan laajentamiseksi ja integroimiseksi läntisille markkinoille on pidettävä poikkeuksellisen oma-aloitteisena ja ahkerana. Hänen toimintansa voi myös nähdä vastapainona ajanjakson yleiselle kehitystrendille, Suomen idänkaupan voimakkaalle kasvulle. Tutkielma täydentää siten kuvaa Suomen ulko- ja kauppapolitiikasta kylmän sodan alkuvuosina sekä Suomen ja Yhdysvaltain suhteiden kehityksestä 1950-luvulla.

Psychiatric manifestations of sporadic Creutzfeldt-Jakob disease simulate depressive disorder with psychotic symptoms

Creutzfeldt–Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a broad spectrum of early clinical manifestations, comprising neurological and psychiatric symptoms. The authors report the case of a patient admitted with a diagnosis of depressive disorder with psychotic symptoms, with post-mortem confirmation of CJD and discuss how CJD’s clinical heterogeneity can lead to misdiagnosis of the disease. Despite CJD’s unique pathogenesis, its kaleidoscopic presentation justifies the integrated investigation of patients with psychiatric symptoms, avoiding restrictive diagnosis.

Effect of host genotype and prion strain on the phenotypic heterogeneity of Creutzfeldt-Jakob disease: the peripheral nervous system involvement and the spectrum of the genetic form

The first study was designed to assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD). To this aim, we reviewed medical records of 117 sCJDVV2, 65 sCJDMV2K, and 121 sCJDMM(V)1 subjects for symptoms/signs and neurophysiological data. We looked for the presence of PrPSc in postmortem PNS samples from 14 subjects by western blotting and real-time quaking-induced conversion (RT-QuIC) assay. Seventy-five (41.2%) VV2-MV2K patients, but only 11 (9.1%) MM(V)1, had symptoms/signs suggestive of PNS involvement and neuropathy was documented in half of the VV2-MV2K patients tested. RT-QuIC was positive in all PNS samples, whereas western blotting detected PrPSc in the sciatic nerve in only one VV2 and one MV2K. These results support the conclusion that peripheral neuropathy, likely related to PrPSc deposition, belongs to the phenotypic spectrum of sCJDMV2K and VV2, the two variants linked to the V2 strain. The second study aimed to characterize the genetic/molecular determinants of phenotypic variability in genetic CJD (gCJD). To this purpose, we compared 157 cases of gCJD to 300 of sCJD. We analyzed: demographic aspects, neurological symptoms/signs, histopathologic features and biochemical characteristics of PrPSc. The results strongly indicated that the clinicopathological phenotypes of gCJD largely overlap with those of sCJD and that the genotype at codon 129 in cis with the mutation (i.e. haplotype) contributes more than the latter to the disease phenotype. Some mutations, however, cause phenotypic variations including haplotype-specific patterns of PrPSc deposition such as the “dense” synaptic pattern (E200K-129M), the intraneuronal dots (E200K-129V), and the linear stripes perpendicular to the surface in the molecular layer of cerebellum (OPRIs-129M). Overall, these results suggest that in gCJD PRNP mutations do not cause the emergence of novel prion strains, but rather confer increased susceptibility to the disease in conjunction with “minor” clinicopathological variations.