caa a22 4500 465753647 CHVBK 20180323111841.0 cr unu---uuuuu 170327e19901101xx s 000 0 eng 10.1007/BF01625735 doi (NATIONALLICENCE)springer-10.1007/BF01625735 Creutzfeldt-Jakob disease with severe involvement of cerebral white matter and cerebellum [Elektronische Daten] [J. Berciano, M. Berciano, J. Polo, J. Figols, J. Ciudad, M. Lafarga] Summary: We describe a patient with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Postmortem examination revealed the characteristic lesions of CJD in the grey matter and profound white matter involvement was seen with immunocytochemical techniques. Ultrastructural white matter lesions were identical to those described in experimentally transmitted CJD. There was marked loss of cerebellar granule cells with virtual disappearance of parallel fibres, but Purkinje cells were only slightly reduced. Electron microscopic studies revealed extensive degenerative changes including cytoplasmic vacuoles in both cell types. Silver methods disclosed massive impregnation of white matter and striking abnormalities of Purkinje cells consisting of hypertrophy and flattening of thick dendritic branches, reduction in the number of terminal branchlets, segmentary loss of spines and polymorphic spines. These findings show the extensive involvement of all three cerebellar cortical layers and the reactive plasticity of Purkinje cells to deafferentiation. They favour the hypothesis that demyelination represents a primary lesion of the white matter. Springer-Verlag, 1990 Creutzfeldt-Jakob disease nationallicence Cerebellar de-generation nationallicence Leucoencephalopathy nationallicence Berciano J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut Berciano M. Department of Anatomy and Cell Biology, University of Cantabria, Santander, Spain aut Polo J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut Figols J. Service of Neuropathology, National Hospital "Marqués de Valdecilla”, Santander, Spain aut Ciudad J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut Lafarga M. Department of Anatomy and Cell Biology, University of Cantabria, Santander, Spain aut Virchows Archiv A Springer-Verlag 417/6(1990-11-01), 533-538 0174-7398 417:6<533 1990 417 428 https://doi.org/10.1007/BF01625735 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01625735 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Berciano J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut NATIONALLICENCE 700 1- Berciano M. Department of Anatomy and Cell Biology, University of Cantabria, Santander, Spain aut NATIONALLICENCE 700 1- Polo J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut NATIONALLICENCE 700 1- Figols J. Service of Neuropathology, National Hospital "Marqués de Valdecilla”, Santander, Spain aut NATIONALLICENCE 700 1- Ciudad J. Service of Neurology, National Hospital "Marqués de Valdecilla”, 39008, Santander, Spain aut NATIONALLICENCE 700 1- Lafarga M. Department of Anatomy and Cell Biology, University of Cantabria, Santander, Spain aut NATIONALLICENCE 773 0- Virchows Archiv A Springer-Verlag 417/6(1990-11-01), 533-538 0174-7398 417:6<533 1990 417 428 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer