Atypical Parkinsonism and Frontotemporal Dementia – Clinical, Pathological and Genetic Aspects

Authors: K. Menšíková 1;  L. Tučková 2;  P. Kaňovský 1
Authors‘ workplace: Neurologická klinika LF UP a FN Olomouc 1;  Ústav klinické a molekulární patologie, LF UP a FN Olomouc 2
Published in: Cesk Slov Neurol N 2016; 79/112(3): 275-286
Category: Review Article


Frontotemporal dementia (FTD) is a heterogeneous clinical syndrome including behavioural variant FTD (bvFTD) and primary progressive aphasia (PPA), which are clinically characterized by behavioural changes and impairment of executive functions and speech. Behavioural changes and speech disorders are often accompanied by motor symptomatology, including especially parkinsonism and/or symptoms of the upper and lower motor neuron involvement. These symptoms may be present in various combinations, thereby creating a very wide spectrum of clinical entities, some of which are precisely genetically and pathologically defined. Besides these few defined entities, there is a number of variable clinical phenotypes consisting of different combinations of behavioural and personality changes, speech disorders and symptoms of typical and atypical parkinsonism associated with different mutations and histopathologic findings. These phenotypes cannot be clearly classified according to their clinical picture and instead, they are placed somewhere within the dementia-parkinsonism continuum. In our paper, we present an overview of the current state of knowledge of possible clinical manifestations of parkinsonism and FTD and their genetic and pathological aspects.

Key words:
frontotemporal dementia – primary progressive aphasia – progressive supranuclear palsy – corticobasal syndrome – frontotemporal lobar degenerations

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.


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Paediatric neurology Neurosurgery Neurology

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Czech and Slovak Neurology and Neurosurgery

Issue 3

2016 Issue 3

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