Universität zu Köln

Nass, R. D., Meister, I. G., Haupt, W. F. and Fink, G. R. (2012). ALS and Frontotemporal Dementia - Case Report and Review of the Literature. Forschritte Neurol. Psychiatr., 80 (12). S. 711 - 720. STUTTGART: GEORG THIEME VERLAG KG. ISSN 1439-3522

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Abstract

The occurrence of cognitive decline in amyotrophic lateral sclerosis (ALS), especially in the form of frontotemporal dementia (FTD), has been described previously. Recent molecular biology and histopathology data suggest that both ALS and FTD may share common pathological pathways and may present two phenotypes of the same proteinopathy. The underlying pathophysiological mechanism may be defective RNA- and DNA-modulation, mediated by the proteins TDP43 and FUS. These findings are suggestive of a new disease category of TDP43-proteinopathies, which include ALS, FTD and overlap syndromes. While about half of the FTD cases are associated with TDP43-deposits, tau is found in the other half. A significant clinical overlap to other tauopathies exists here as well, for instance with corticobasal degeneration. In this paper, we present a case report and review the clinical spectrum and current pathogenetic concepts of FTD.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Nass, R. D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Meister, I. G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Haupt, W. F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Fink, G. R. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-477189
DOI:	10.1055/s-0032-1325604
Journal or Publication Title:	Forschritte Neurol. Psychiatr.
Volume:	80
Number:	12
Page Range:	S. 711 - 720
Date:	2012
Publisher:	GEORG THIEME VERLAG KG
Place of Publication:	STUTTGART
ISSN:	1439-3522
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language AMYOTROPHIC-LATERAL-SCLEROSIS; LOBAR DEGENERATION; COGNITIVE IMPAIRMENT; HEXANUCLEOTIDE REPEAT; DIAGNOSTIC-CRITERIA; CEREBROSPINAL-FLUID; QUANTITATIVE EEG; CSF BIOMARKERS; MUTATIONS; TDP-43 Multiple languages Clinical Neurology; Psychiatry Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/47718