Early frontotemporal dementia targets neurons unique to apes and humans. Export

@article{citeulike:3859604, abstract = {OBJECTIVE: Frontotemporal dementia (FTD) is a neurodegenerative disease that erodes uniquely human aspects of social behavior and emotion. The illness features a characteristic pattern of early injury to anterior cingulate and frontoinsular cortex. These regions, though often considered ancient in phylogeny, are the exclusive homes to the von Economo neuron (VEN), a large bipolar projection neuron found only in great apes and humans. Despite progress toward understanding the genetic and molecular bases of FTD, no class of selectively vulnerable neurons has been identified. METHODS: Using unbiased stereology, we quantified anterior cingulate VENs and neighboring Layer 5 neurons in FTD (n = 7), Alzheimer's disease (n = 5), and age-matched nonneurological control subjects (n = 7). Neuronal morphology and immunohistochemical staining patterns provided further information about VEN susceptibility. RESULTS: FTD was associated with early, severe, and selective VEN losses, including a 74\% reduction in VENs per section compared with control subjects. VEN dropout was not attributable to general neuronal loss and was seen across FTD pathological subtypes. Surviving VENs were often dysmorphic, with pathological tau protein accumulation in Pick's disease. In contrast, patients with Alzheimer's disease showed normal VEN counts and morphology despite extensive local neurofibrillary pathology. INTERPRETATION: VEN loss links FTD to its signature regional pattern. The findings suggest a new framework for understanding how evolution may have rendered the human brain vulnerable to specific forms of degenerative illness.}, author = {Seeley, W. W. and Carlin, D. A. and Allman, J. M. and Macedo, M. N. and Bush, C. and Miller, B. L. and Dearmond, S. J.}, citeulike-article-id = {3859604}, citeulike-linkout-0 = {http://dx.doi.org/10.1002/ana.21055}, citeulike-linkout-1 = {http://view.ncbi.nlm.nih.gov/pubmed/17187353}, citeulike-linkout-2 = {http://www.hubmed.org/display.cgi?uids=17187353}, doi = {10.1002/ana.21055}, issn = {0364-5134}, journal = {Annals of neurology}, keywords = {acc, dementia, neuroscience, psychiatry, spindlecells}, month = {December}, number = {6}, pages = {660--667}, posted-at = {2009-01-08 09:17:35}, priority = {2}, title = {Early frontotemporal dementia targets neurons unique to apes and humans.}, url = {http://dx.doi.org/10.1002/ana.21055}, volume = {60}, year = {2006} }

TY - JOUR ID - citeulike:3859604 L3 - citeulike-article-id:3859604 N2 - OBJECTIVE: Frontotemporal dementia (FTD) is a neurodegenerative disease that erodes uniquely human aspects of social behavior and emotion. The illness features a characteristic pattern of early injury to anterior cingulate and frontoinsular cortex. These regions, though often considered ancient in phylogeny, are the exclusive homes to the von Economo neuron (VEN), a large bipolar projection neuron found only in great apes and humans. Despite progress toward understanding the genetic and molecular bases of FTD, no class of selectively vulnerable neurons has been identified. METHODS: Using unbiased stereology, we quantified anterior cingulate VENs and neighboring Layer 5 neurons in FTD (n = 7), Alzheimer's disease (n = 5), and age-matched nonneurological control subjects (n = 7). Neuronal morphology and immunohistochemical staining patterns provided further information about VEN susceptibility. RESULTS: FTD was associated with early, severe, and selective VEN losses, including a 74% reduction in VENs per section compared with control subjects. VEN dropout was not attributable to general neuronal loss and was seen across FTD pathological subtypes. Surviving VENs were often dysmorphic, with pathological tau protein accumulation in Pick's disease. In contrast, patients with Alzheimer's disease showed normal VEN counts and morphology despite extensive local neurofibrillary pathology. INTERPRETATION: VEN loss links FTD to its signature regional pattern. The findings suggest a new framework for understanding how evolution may have rendered the human brain vulnerable to specific forms of degenerative illness. IS - 6 JF - Annals of neurology SN - 0364-5134 EP - 667 TI - Early frontotemporal dementia targets neurons unique to apes and humans. VL - 60 SP - 660 KW - acc KW - dementia KW - neuroscience KW - psychiatry KW - spindlecells AU - Seeley, WW AU - Carlin, DA AU - Allman, JM AU - Macedo, MN AU - Bush, C AU - Miller, BL AU - Dearmond, SJ PY - 2006/12// UR - http://dx.doi.org/10.1002/ana.21055 ER -