Classification and Description of Frontotemporal Dementias
| Autor: | David Neary, J S Snowden, D. M. A. Mann |
|---|---|
| Rok vydání: | 2006 |
| Předmět: |
Male
Pathology medicine.medical_specialty Tau pathology Semantic dementia General Biochemistry Genetics and Molecular Biology Diagnosis Differential History and Philosophy of Science Aphasia mental disorders medicine Humans Motor Neuron Disease Aged General Neuroscience Frontotemporal lobar degeneration Middle Aged medicine.disease Temporal Lobe Frontal Lobe Mutation Etiology Dementia Female Tauopathy medicine.symptom Focal degeneration Psychology Neuroscience Frontotemporal dementia |
| Zdroj: | Annals of the New York Academy of Sciences. 920:46-51 |
| ISSN: | 1749-6632 0077-8923 |
| DOI: | 10.1111/j.1749-6632.2000.tb06904.x |
| Popis: | A number of distinct clinical syndromes have been described that are associated with focal degeneration of the frontal and temporal lobes and have a non-Alzheimer pathology. The nosological status of frontotemporal lobar degeneration (FTLD) has been a matter of controversy, in view of the diversity of clinical manifestations and distribution and nature of histopathological change. This paper describes the major clinical syndromes of frontotemporal dementia, progressive aphasia, and semantic dementia; it discusses their underlying pathologies and considers their molecular status. Common histopathological changes are demonstrated across the three clinical syndromes, highlighting the link between these clinical disorders. It has been suggested that these disorders should be regarded as tauopathies on the basis of the tau pathology seen in a number of cases and the mutations in the tau gene in some familial cases. However, in a series of 47 consecutive autopsy series of FTLD, only 36% had tau pathology and 10% mutations in the tau gene, suggesting that FTLD does not constitute a unitary etiological disorder and that its characterization as a tauopathy may be potentially misleading. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text