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      Dementia and Art: Neuronal Intermediate Filament Inclusion Disease and Dissolution of Artistic Creativity

      case-report

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          Abstract

          The paper presents a new case of neuronal intermediate filament inclusion disease (NIFID), a recently described new variant of early-onset frontotemporal dementia. Documented with repetitive brain images, morphologically proven cases additionally endorse evolving the clinical and pathological phenotype of NIFID. For the first time the paper describes the probable influence of NIFID on the artistic creativity of an accomplished artist showing rapid dissolution of artistic talent.

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          Most cited references14

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          Portraits of artists: emergence of visual creativity in dementia.

          As a uniquely human talent that has evolved from prehistoric cave paintings, art draws on many brain areas responsible for various cognitive processes. The pattern of degeneration in dementia leads to predictable changes in art. The visuospatial deficits in Alzheimer disease lead to less precision and attention to spatial relationships. In some cases of frontotemporal dementia, artistic creativity appears anew as the disease develops. The artwork is approached in a compulsive manner and is often realistic or surrealistic in style. Art in the context of dementia provides a unique window into the cognitive processes of various brain regions and an opportunity for rehabilitation.
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            Neurofilament inclusion body disease: a new proteinopathy?

            We describe four cases of a new clinicopathological entity presenting with either a frontotemporal dementia or corticobasal degeneration syndrome with a mean age of onset of 45 years (range 41-50) characterized pathologically by deposition of neurofilament proteins. All four patients had a rapidly progressive course and have become mute and non-ambulatory, and three have died after mean illness duration of only 3 years (range 2 1/2 -4). Both structural (MRI) and functional (PET and SPECT) imaging demonstrated frontal and temporal lobe and basal ganglia involvement. Gross neuropathological examination in the three deceased patients (the fourth patient, still alive, was diagnosed by brain biopsy) revealed changes affecting predominantly the frontal and temporal cortices, basal ganglia and brainstem. There was superficial linear spongiosis affecting the frontal lobes in all three autopsied patients, and severe caudate atrophy was noted in two of them and demonstrated on MRI in the living patient. On routine staining, there were numerous intracytoplasmic inclusions, which ranged from eosinophilic to basophilic. Some had a clearly defined basophilic margin, while others were granular with a hyaline core. With modified Bielschowsky silver technique, a small number of the inclusions were intensely stained. Inclusions were not labelled with other silver stains. Immuno histochemistry revealed that the inclusions were immunoreactive with antibodies to neurofilament heavy and light chain subunits and to ubiquitin, but not with antibodies to tau and alpha-synuclein. These neurofilament- and ubiquitin-positive inclusions were widespread, specific to neurons and occasionally intranuclear. The frequency and distribution of the inclusions and the silver and immunohistochemical profiles in these four cases is novel and has not been described in detail before. We propose the term neurofilament inclusion body disease for this entity.
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              Artistic Creativity, Style and Brain Disorders

              The production of novel, motivated or useful material defines creativity, which appears to be one of the higher, specific, human brain functions. While creativity can express itself in virtually any domain, art might particularly well illustrate how creativity may be modulated by the normal or pathological brain. Evidence emphasizes global brain functioning in artistic creativity and output, but critical steps which link perception processing to execution of a work, such as extraction-abstraction, as well as major developments of non-esthetic values attached to art also underline complex activation and inhibition processes mainly localized in the frontal lobe. Neurological diseases in artists provide a unique opportunity to study brain-creativity relationships, in particular through the stylistic changes which may develop after brain lesion.
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                Author and article information

                Journal
                ENE
                Eur Neurol
                10.1159/issn.0014-3022
                European Neurology
                S. Karger AG
                0014-3022
                1421-9913
                2007
                March 2007
                10 January 2007
                : 57
                : 3
                : 137-144
                Affiliations
                aClinics of Neurology and Neurosurgery, Vilnius University Faculty of Medicine, bNational Center of Pathology and cDepartment of Psychiatry, Vilnius Republical Hospital of Psychiatry, Vilnius, Lithuania; dDepartment/Institute of Pathology, Rikshospitalet-Radiumhospitalet HF, Oslo, Norway
                Article
                98464 Eur Neurol 2007;57:137–144
                10.1159/000098464
                17213719
                93f101f0-53cc-4087-b1ea-8fa5423172e3
                © 2007 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
                : 31 May 2006
                : 20 October 2006
                Page count
                Figures: 8, References: 23, Pages: 8
                Categories
                Neurology and Art

                Geriatric medicine,Neurology,Cardiovascular Medicine,Neurosciences,Clinical Psychology & Psychiatry,Public health
                Artistic creativity,Neuronal intermediate filament inclusion disease,Frontotemporal dementia

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