Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions
| Autor: | Akira Tamaoka, Osamu Yokota, Haruhiko Akiyama, Akihide Mochizuki, Seishi Terada, Hidetoshi Yoshida, Mitsuru Kawamura, Kuniaki Tsuchiya, Hideki Ishizu, Shigetoshi Kuroda, Saburo Yagishita, Osamu Matsubara, Tetsuaki Arai |
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| Rok vydání: | 2009 |
| Předmět: |
Male
Pathology medicine.medical_specialty Semantic dementia Pathology and Forensic Medicine Diagnosis Differential Cellular and Molecular Neuroscience Pick Disease of the Brain mental disorders medicine Humans Corticobasal degeneration Motor Neuron Disease Retrospective Studies Brain Chemistry Inclusion Bodies Neurons Temporal cortex Language Disorders Movement Disorders Cell Death Ubiquitin business.industry Parkinsonism Brain nutritional and metabolic diseases Frontotemporal lobar degeneration Middle Aged medicine.disease nervous system diseases DNA-Binding Proteins Spinal Cord Nerve Degeneration Corticospinal tract Dementia Female Pick's disease Neurology (clinical) business Neuroglia Frontotemporal dementia |
| Zdroj: | Acta Neuropathologica. 117:429-444 |
| ISSN: | 1432-0533 0001-6322 |
| DOI: | 10.1007/s00401-009-0493-4 |
| Popis: | Although frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick's disease are common pathological substrates in sporadic FTLD, clinical differentiation of these diseases is difficult. We performed a retrospective review of medical records and semiquantitative examination of neuronal loss of 20 sporadic FTLD-TDP and 19 Pick's disease cases. Semantic dementia as the first syndrome developed only in FTLD-TDP patients. Impaired speech output in the early stage was five times more frequent in Pick's disease than in FTLD-TDP. The total frequency of asymmetric motor disturbances (e.g., parkinsonism, pyramidal signs, and contracture) during the course was significantly more frequent in FTLD-TDP (78%) than in Pick's disease cases (14%). Asymmetric pyramidal signs were found in 7 of 13 FTLD-TDP cases with corticospinal tract degeneration similar to primary lateral sclerosis. Frontotemporal dementia as the first syndrome was noted in both FTLD-TDP (28%) and Pick's disease cases (64%); however, only FTLD-TDP cases subsequently developed asymmetric motor disturbances, and some of the cases further exhibited hemineglect. Concordant with these clinical findings, degeneration in the temporal cortex, caudate nucleus, putamen, globus pallidus, substantia nigra, and corticospinal tract was significantly more severe in FTLD-TDP, and degeneration in the frontal cortex tended to be more severe in Pick's disease. Given these findings, the initial impairment of semantic memory or comprehension and subsequent asymmetric motor disturbances in sporadic FTLD patients predict sporadic FTLD-TDP rather than Pick's disease, while initial behavioral symptoms or non-fluent aphasia without subsequent asymmetric motor disturbances predict Pick's disease rather than sporadic FTLD-TDP. |
| Databáze: | OpenAIRE |
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