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      Frontotemporal Dementia Can Be Distinguished from Alzheimer’s Disease and Subcortical White Matter Dementia by an Anterior-to-Posterior rCBF-SPET Ratio

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          Abstract

          Sixteen patients with frontotemporal dementia (FTD), 27 with early-onset Alzheimer’s disease, 25 with late-onset Alzheimer’s disease, 19 with subcortical white matter dementia (SWD) and 28 normal controls underwent semiquantitative regional cerebral blood flow measurement (rCBF) using single-photon emission tomography (SPET; <sup>99m</sup>Tc-HMPAO) and either computerized tomography (CT) or magnetic resonance imaging (MRI) of the brain. An anterior-to-posterior rCBF-SPET ratio (mesial superior frontal gyrus/medial temporal lobes) was calculated, which significantly separated the FTD group from the other dementia groups and controls with a sensitivity of 87.5% and a specificity of at least 78.6%. CT/MRI was found to be helpful in the differential diagnosis between FTD and SWD. In FTD patients, the mesial superior frontal gyrus, near the polus frontalis, was found to be the region with the most reduced rCBF values.

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          Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis.

          In a longitudinal prospective study of dementia, 158 patients were investigated post mortem. Sixteen patients were classified as frontal lobe dementia (FLD) of non-Alzheimer type and four cases as Pick's disease. Positive heredity for dementia was reported in 50% of these cases compared to 30% in a reference group of patients with Alzheimer's disease (AD). The typical clinical picture in FLD and Pick's disease was that of a slowly progressive dementia, at an early stage dominated by personality change, lack of insight, disinhibition, and later on stereotypy and increased apathy. There was also a progressive dynamic aphasia ending in mutism and amimia. Memory and spatial functions were comparatively spared. Disinhibition, oral/dietary hyperactivity, and echolalia were more consistently found in Pick's disease compared to FLD. The differential diagnosis against AD, cerebrovascular dementia, and other degenerative dementias and against affective disorders and psychotic reactions are discussed.
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            Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology.

            Arne Brun (1987)
            Among 158 cases of organic dementia in a prospective study concerning both psychiatry and regional cerebral blood flow there were 26 cases with a mainly frontal or fronto-temporal dementia. Careful neuropathological investigation disclosed 20 cases of a mainly frontal or fronto-temporal grey matter degeneration, in four of them compatible with Pick's disease (2.5%) whereas 16 cases (10%) appeared to form a separate group without histological Alzheimer features and therefore named 'frontal lobe degeneration of non-Alzheimer type' (FLD). The remaining group of dementias of a clinically similar type proved to consist of cases of Jakob-Creutzfeldt's and Alzheimer's disease with frontal predominance and also a case of normal frontal cortex with a projected dysfunction caused by bilateral thalamic infarctions. Also other similar conditions are accounted for from the literature. The validity of the pathological changes described here, particularly with regard to their severity and regional distribution as well as their tendency to spare certain areas is attested by the clinical picture including neuropsychiatric symptoms and the regional cerebral blood flow pattern, both consistently producing the picture of a frontal and fronto-temporal disease of a progressive degenerative type. These features are dealt with in the following papers by Gustafson (1987) and Risberg (1987). FLD is in some morphological respects similar to other dementing disorders such as the ALS dementia complex and progressive subcortical gliosis, though with both clinical and clear-cut pathoanatomical differences. For the time being it seems safest to conclude that we are faced with a hitherto not fully recognized if not a new type of dementia caused by 'simple' neuronal degeneration of mainly the frontal or frontal and temporal lobes. It makes up about 10% of organic dementias, a figure that would be higher in purely clinical classifications due to the admixture of other frontal lobe disorders or frontally projected dysfunction clinically simulating FLD of the pathoanatomical type here described.
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              Reduced temporal lobe blood flow in alzheimer's disease

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                Author and article information

                Journal
                DEM
                Dement Geriatr Cogn Disord
                10.1159/issn.1420-8008
                Dementia and Geriatric Cognitive Disorders
                S. Karger AG
                1420-8008
                1421-9824
                2000
                October 2000
                09 August 2000
                : 11
                : 5
                : 275-285
                Affiliations
                aInstitute of Clinical Neuroscience, Göteborg University, Göteborg, and bDepartment of Psychogeriatrics, University of Lund, Sweden
                Article
                17250 Dement Geriatr Cogn Disord 2000;11:275–285
                10.1159/000017250
                10940679
                603e80dc-9205-400b-b19b-5a181bff9e42
                © 2000 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Page count
                Figures: 3, References: 56, Pages: 11
                Categories
                Original Research Article

                Geriatric medicine,Neurology,Cardiovascular Medicine,Neurosciences,Clinical Psychology & Psychiatry,Public health
                Vascular dementia,Alzheimer’s disease,Single-photon emission tomography,Subcortical white matter dementia,Regional cerebral blood flow,Frontotemporal dementia

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