Frontal Dementias
Etiological, Clinical, Therapeutical and Pathological Aspects
3rd International Conference, Lund, Sweden, August 27-29, 1998
Guest Edtitors: Lars Gustafson, Lund; Arne Brun, Lund

Paper

Neuropathology of Chromosome-17-Linked Dementia
Anders A.F. Sima^a,c, Constance D'Amato^a, Norman L. Foster^b

Departments of
^aPathology and
^bNeurology, University of Michigan, Ann Arbor, Mich., and
^cDepartments of Pathology and Neurology, Wayne State University, Detroit, Mich., USA

Address of Corresponding Author

Dement Geriatr Cogn Disord 1999;10:61-63 (DOI: 10.1159/000051215)

  Key Words

• Dementia
• Chromosome 17
• Frontotemporal atrophy
• Neuronal inclusions
• Glial cytoplasmic inclusions

  Abstract

We describe the characteristic neuropathologic changes in chromosome-17-linked dementia. Identified cases to date demonstrate a uniform pathology consisting of neuronal loss, gliosis and vacuolization of frontal, anterior cingulate and temporal cortex and occipital association areas. Similar changes are present in substantia nigra, ventral striatum and amygdala. Hippocampus itself is spared, whereas the afferent perforant tract is consistently affected. Characteristic neuronal inclusions consisting of lattice-like neurofilaments are present in subcortical nuclei and glial cytoplasmic tau-positive inclusions are present throughout the white matter. These pathologic changes appear to be unique for chromosome-17-linked dementia.

  Author Contacts

Anders A.F. Sima, MD, PhD
Department of Pathology, Wayne State University
540 E Canfield Ave, Detroit, MI 48201 (USA)
Tel. +1 313 577 1150, Fax +1 313 577 0057
E-Mail asima@med.wayne.edu

  Article Information

Number of Print Pages : 3
Number of Figures : 2, Number of Tables : 1, Number of References : 14