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      Clinical Subtypes of Alzheimer’s Disease

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          Abstract

          Alzheimer''s disease (AD) can present as a variety of clinical profiles. Although the most common presentation is that of a progressive amnestic disorder with subsequent involvement of other cognitive functions and personality alterations, there are numerous other clinical profiles. AD can present as a focal cortical degenerative syndrome with the clinical features dependent on the regions of the brain involved. Some of these syndromes include disturbances of language, visuospatial skills, attentional functions, executive processes and praxis. The neuropathological substrate of these disorders is variable and can include AD. Recently, the Lewy body variant of AD has been described. Finally, other modifying features that affect the progression of AD, such as extrapyramidal symptoms and myoclonus, are also discussed. Although the progressive amnestic form of AD is the most common presentation, other variations on the clinical syndrome can be important to identify because they may have implications for prognosis and treatment.

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          Most cited references 6

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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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              Senile dementia of Lewy body type

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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
                978-3-8055-6762-6
                978-3-318-00351-2
                1420-8008
                1421-9824
                1998
                August 1998
                23 October 1998
                : 9
                : Suppl 3
                : 16-24
                Affiliations
                Mayo Alzheimer’s Disease Center, Department of Neurology, Mayo Clinic,Rochester, Minn., USA
                Article
                51199 Dement Geriatr Cogn Disord 1998;9(suppl 3):16–24
                10.1159/000051199
                9853198
                © 1998 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.

                Page count
                Figures: 5, References: 67, Pages: 9
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