The neuropathology of chromosome 17-linked dementia
dc.contributor.author | Sima, Anders A. F. | en_US |
dc.contributor.author | Defendini, R. | en_US |
dc.contributor.author | Keohane, C. | en_US |
dc.contributor.author | D'Amato, Constance J. | en_US |
dc.contributor.author | Foster, Norman L. | en_US |
dc.contributor.author | Parchi, P. | en_US |
dc.contributor.author | Gambetti, P. | en_US |
dc.contributor.author | Lynch, T. | en_US |
dc.contributor.author | Wilhelmsen, Kirk C. | en_US |
dc.date.accessioned | 2007-04-06T18:55:39Z | |
dc.date.available | 2007-04-06T18:55:39Z | |
dc.date.issued | 1996-06 | en_US |
dc.identifier.citation | Sima, A. A. F.; Defendini, R.; Keohane, C.; D'Amato, C.; Foster, N. L.; Parchi, P.; Gambetti, P.; Lynch, T.; Wilhelmsen, K. C. (1996)."The neuropathology of chromosome 17-linked dementia." Annals of Neurology 39(6): 734-743. <http://hdl.handle.net/2027.42/50360> | en_US |
dc.identifier.issn | 0364-5134 | en_US |
dc.identifier.issn | 1531-8249 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/50360 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=8651645&dopt=citation | en_US |
dc.description.abstract | We recently described a family with chromosome 17-linked dementia, characterized clinically by disinhibition-dementia parkinsonism-amyotrophy complex. We report now the neuropathology of 6 affected family members. This included semiquantitative scoring of neuronal loss, gliosis, and spongiosis and immunocytochemical and ultrastructural characterization of neuronal and glial inclusions. The changes consisted of circumscribed neuronal loss, gliosis, and spongiosis of limbic neocortical areas and frontal, temporal, and occipital association areas. Similar changes were present in subcortical nuclei, most severe in the substantia nigra, but also involved the ventral striatum and amygdala. The hippocampus was spared except for degeneration of the afferent perforant tract, secondary to entorhinal nerve cell loss. Hgyrophilic neuronal inclusions, with a characteristic immunocytochemical profile, were found in brainstem nuclei, hypothalamus, and basal ganglia. Ultrastructurally, in 3 patients these inclusions showed hitherto undescribed abnormally assembled filaments. Glial cytoplasmic inclusions were widespread in white matter structures. Immunocytochemistry failed to demonstrate the protease-resistant prion protein. The pathology appears to be unique, involving various cortical and subcortical structures, and is consistent with the clinical findings of Kliiver-Bucy-like syndrome, parkinsonism, and frontal lobe dementia. For this entity we suggest the term “chromosome 17- linked dementia”. | en_US |
dc.format.extent | 1319899 bytes | |
dc.format.extent | 3118 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.publisher | Wiley Subscription Services, Inc., A Wiley Company | en_US |
dc.subject.other | Life and Medical Sciences | en_US |
dc.subject.other | Neuroscience, Neurology, and Psychiatry | en_US |
dc.title | The neuropathology of chromosome 17-linked dementia | en_US |
dc.type | Article | en_US |
dc.rights.robots | IndexNoFollow | en_US |
dc.subject.hlbsecondlevel | Psychiatry | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Departments of Pathology, University of Michigan Medical Center, Ann Arbor, MI ; Departments of Internal Medicine, University of Michigan Medical Center, Ann Arbor, MI ; University of Michigan Medical center, 1331 E. Ann street, Box 0580, Ann Arbor, MI 48103 | en_US |
dc.contributor.affiliationum | Departments of Pathology, University of Michigan Medical Center, Ann Arbor, MI | en_US |
dc.contributor.affiliationum | Departments of Neurology, University of Michigan Medical Center, Ann Arbor, MI | en_US |
dc.contributor.affiliationother | Division of Neuropathology, Columbia-Presbyterian Medical Center, New York, NY | en_US |
dc.contributor.affiliationother | Department of Pathology, Cork University Hospital, Wilton, Cork, Ireland | en_US |
dc.contributor.affiliationother | Institute of Pathology, Case Western Reserve University, Cleveland, OH | en_US |
dc.contributor.affiliationother | Institute of Pathology, Case Western Reserve University, Cleveland, OH | en_US |
dc.contributor.affiliationother | Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY | en_US |
dc.contributor.affiliationother | Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY | en_US |
dc.identifier.pmid | 8651645 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/50360/1/410390609_ftp.pdf | en_US |
dc.identifier.doi | http://dx.doi.org/10.1002/ana.410390609 | en_US |
dc.identifier.source | Annals of Neurology | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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