Zerr, I., Kallenberg, K., Summers, D. M., Romero, C., Taratuto, A., Heinemann, U., Breithaupt, M., Varges, D., Meissner, B., Ladogana, A., Schuur, M., Haik, S., Collins, S. J., Jansen, G. H., Stokin, G. B., Pimentel, J., Hewer, E., Collie, D., Smith, P., Roberts, H., Brandel, J. P., van Duijn, C., Pocchiari, M., Begue, C., Cras, P., Will, R. G., Sanchez-Juan, P.

Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. […]

Tikka, S., Mykkanen, K., Ruchoux, M.-M., Bergholm, R., Junna, M., Poyhonen, M., Yki-Jarvinen, H., Joutel, A., Viitanen, M., Baumann, M., Kalimo, H.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary subcortical vascular dementia. It is caused by mutations in NOTCH3 gene, which encodes a large transmembrane receptor Notch3. The key pathological finding is the accumulation of granular osmiophilic material (GOM), which contains extracellular domains of Notch3, on degenerating vascular smooth […]

Stoeck, K., Sanchez-Juan, P., Gawinecka, J., Green, A., Ladogana, A., Pocchiari, M., Sanchez-Valle, R., Mitrova, E., Sklaviadis, T., Kulczycki, J., Slivarichova, D., Saiz, A., Calero, M., Knight, R., Aguzzi, A., Laplanche, J.-L., Peoc'h, K., Schelzke, G., Karch, A., van Duijn, C. M., Zerr, I.

To date, cerebrospinal fluid analysis, particularly protein 14-3-3 testing, presents an important approach in the identification of Creutzfeldt-Jakob disease cases. However, one special point of criticism of 14-3-3 testing is the specificity in the differential diagnosis of rapid dementia. The constant observation of increased cerebrospinal fluid referrals in the national surveillance centres over the last […]

Jesso, S., Morlog, D., Ross, S., Pell, M. D., Pasternak, S. H., Mitchell, D. G. V., Kertesz, A., Finger, E. C.

Patients with behavioural variant frontotemporal dementia demonstrate abnormalities in behaviour and social cognition, including deficits in emotion recognition. Recent studies suggest that the neuropeptide oxytocin is an important mediator of social behaviour, enhancing prosocial behaviours and some aspects of emotion recognition across species. The objective of this study was to assess the effects of a […]