Deviance Distraction and Stimulus-Specific Adaptation in the Somatosensory Cortex Reduce with Experience.

Automatic detection of a surprising change in the sensory input is a central element of exogenous attentional control. Stimulus-specific adaptation (SSA) is a potential neuronal mechanism detecting such changes and has been robustly described across sensory modalities and different instances of the ascending sensory pathways. However, little is known about the relationship of SSA to perception.

Developmental dynamics of neurotensin binding sites in the human hypothalamus during the first postnatal year.

The aim of the present study was to determine a detailed mapping of neurotensin (NT) in the human hypothalamus, during the first postnatal year using an in vitro quantitative autoradiography technique and the selective radioligand monoiodo-Tyr3-NT. Ten human postmortem hypothalami obtained from control neonates and infants (aged from 2 h to 1 year of postnatal age) were used. The biochemical kinetics of the binding in all obtained in this study revealed that the binding affinity constants were of high affinity (in the nanomolar range) and did not differ significantly between all cases investigated.

An autoradiographic study of neurotensin receptors in the human hypothalamus.

The aim of the present investigation was to determine a detailed mapping of neurotensin (NT) in the human hypothalamus, the brain region involved in neuroendocrine control. For this, we investigated the presence and the distribution of neurotensin binding sites in the human hypothalamus, using an in vitro quantitative autoradiography technique and the selective radioligand monoiodo-Tyr3-neurotensin (2000Ci/mM). This study was performed on nine adult human postmortem hypothalami.

Ultrastructural characteristics (or evaluation) of Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies or prion diseases.

The authors report on a large series of human prion diseases to establish ultrastructural characteristics that may be useful for their diagnosis. For Creutzfeldt-Jakob disease (CJD and its variant, vCJD) and fatal familial insomnia (FFI) only vacuolation (spongiform change) and the presence of tubulovesicular structures are consistent findings. Other changes, such as the presence of myelinated vacuoles, branching cisternae, neuroaxonal dystrophy, and autophagic vacuoles, were present in different proportions in either CJD or FFI, but they are nonspecific ultrastructural findings that can also occur in other neurodegenerative conditions.

Tubulovesicular structures are a consistent (and unexplained) finding in the brains of humans with prion diseases.

Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS) and Fatal Familial Insomnia (FFI) are slow neurodegenerative disorders classified as transmissible spongiform encephalopathies (TSEs) or prion diseases, which appear in sporadic, hereditary or environmentally acquired forms. Tubulovesicular structures (TVS) are ultrastructural particles of unknown origin and chemical composition found in the brains of both animal and human forms of transmissible spongiform encephalopathies or prion diseases. In this paper, we report the results of a search for TVS in a total of 13 cases of sporadic Creutzfeldt-Jakob disease, three cases of Gerstmann-Sträussler-Scheinker disease, two cases of Fatal Familial Insomnia, and individual cases of familial, iatrogenic, and variant CJD (vCJD).

Interlaboratory comparison of assessments of Alzheimer disease-related lesions: a study of the BrainNet Europe Consortium.

This interlaboratory study evaluated the reproducibility of the assessments of neuritic plaques and neurofibrillary tangles (NFTs)--the hallmark lesions of Alzheimer disease--and compared the staining between the BrainNet Europe centers. To reduce the topography-related inconsistencies in assessments, we used a 2-mm tissue microarray (TMA) technique. The TMA block included 42 core samples taken from 21 paraffin blocks.

A new mutation of the tau gene, G303V, in early-onset familial progressive supranuclear palsy.

Background: Progressive supranuclear palsy (PSP) is a clinicopathological syndrome related to tau deposits and in linkage disequilibrium with tau polymorphisms. Some rare familial PSP cases have been related to tau gene mutations.

Objective: To present the clinical, pathological, and molecular data of one family with early-onset autosomal dominant PSP.

Spinocerebellar ataxia with sensory neuropathy (SCA25).

Spinocerebellar ataxia 25 (SCA25) is a rare form of autosomal dominant cerebellar ataxia associated with a severe sensory neuropathy. Clinical variability ranges from incomplete penetrance at age 61 to a Friedreich ataxia-like syndrome. The responsible locus was mapped to chromosome 2p in a large region of 14 Mbases in a single French kindred.

Autophagy is a part of ultrastructural synaptic pathology in Creutzfeldt-Jakob disease: a brain biopsy study.

Ultrastructural correlates of synaptic and dendritic spines loss have never been studied in detail in human transmissible spongiform encephalopathies (TSEs)-Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) disease and fatal familial insomnia (FFI). In this paper, we describe synaptic alterations as found in brain biopsies from Creutzfeldt-Jakob disease and fatal familial insomnia patients. Our material consisted of brain biopsies obtained by open surgery from one FFI case, one case of variant Creutzfeldt-Jakob disease (vCJD), seven cases of sporadic Creutzfeldt-Jakob disease (sCJD) and one case of iatrogenic (human growth hormone) Creutzfeldt-Jakob disease (iCJD).

Quantitative study of spongiform change in putamen of 24 cases of Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease (CJD) is characterized by 4 main neuropathological lesions: spongiform change, neuronal loss, astrocytic gliosis, and accumulation of pathological prion protein (PrPsc), which is partially protease-resistant (PrPres). This study focused on spongiform change (SC) in the putamen. Because SC varies from case to case, we investigated whether its quantification could provide relevant criteria to discriminate types of PrPres in CJD.

Dumbbell-shaped epidural capillary hemangioma.

We report a case of a purely epidural capillary hemangioma of the thoracic spine with foraminal extension. Epidural hemangiomas are rare; only a few cases of dumbbell-shaped ones have been reported, and all were cavernous. MR imaging showed characteristic findings of a capillary hemangioma, which are also consistent with other epidural lesions such as neuromas or meningiomas.

Immunohistochemical localization of 14.3.3 zeta protein in amyloid plaques in human spongiform encephalopathies.

The localization of 14.3.3 proteins was studied in different subtypes of brain amyloid plaques.

Chordoid glioma of the third ventricle: a report of two new cases, with further evidence supporting an ependymal differentiation, and review of the literature.

The term chordoid glioma of the third ventricle was first used to describe a rare and slowly growing neoplasm of uncertain histogenesis, with chordoid appearance, occurring preferentially in middle-aged women. Herein we report two additional examples of this novel entity together with a literature review based on the 25 cases previously published. Our review fully confirms the strikingly stereotyped clinical, neuroradiologic, and pathologic features of this unique tumor.

Orofacial dyskinesias in a patient with primary biliary cirrhosis: a clinicopathological case report and review.

We describe the pathological and clinical aspects, including video and radiological magnetic resonance imaging, of a case of chronic acquired hepatocerebral degeneration with orofacial dyskinesias in relation to a primary biliary cirrhosis. We provide a review of the literature on this subject.