Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic

Background: An epidemiological study conducted over four years revealed increased prevalence of neurodegenerative parkinsonism in a small, isolated region (10 villages, with a combined population of 8664, with approx. 2927 over 50 years of age) of south-eastern Moravia, Czech Republic. The aim of th...

Full description

Bibliographic Details
Main Authors: Katerina Mensikova, Marek Godava, Petr Kanovsky, Pavel Otruba, Michaela Kaiserova, Miroslav Vastik, Lenka Mikulicova, Tereza Bartonikova, Radek Vrtel, Radek Vodicka, Sandra Kurcova, Petr Jugas, Josef Ovecka, Ludmila Sachova, Frantisek Dvorsky
Format: Article
Language:English
Published: Palacký University Olomouc, Faculty of Medicine and Dentistry 2016-03-01
Series:Biomedical Papers
Subjects:
Online Access:https://biomed.papers.upol.cz/artkey/bio-201601-0023_Familial_autosomal-dominant_neurodegenerative_parkinsonism_with_cognitive_deterioration_spanning_five_generati.php
_version_ 1818325204726710272
author Katerina Mensikova
Marek Godava
Petr Kanovsky
Pavel Otruba
Michaela Kaiserova
Miroslav Vastik
Lenka Mikulicova
Tereza Bartonikova
Radek Vrtel
Radek Vodicka
Sandra Kurcova
Petr Jugas
Josef Ovecka
Ludmila Sachova
Frantisek Dvorsky
author_facet Katerina Mensikova
Marek Godava
Petr Kanovsky
Pavel Otruba
Michaela Kaiserova
Miroslav Vastik
Lenka Mikulicova
Tereza Bartonikova
Radek Vrtel
Radek Vodicka
Sandra Kurcova
Petr Jugas
Josef Ovecka
Ludmila Sachova
Frantisek Dvorsky
author_sort Katerina Mensikova
collection DOAJ
description Background: An epidemiological study conducted over four years revealed increased prevalence of neurodegenerative parkinsonism in a small, isolated region (10 villages, with a combined population of 8664, with approx. 2927 over 50 years of age) of south-eastern Moravia, Czech Republic. The aim of this study was to obtain more detailed information on the medical history of the relatives of individuals with confirmed parkinsonism in an isolated rural population in south-eastern Moravia, Czech Republic. Methods: We did detailed genealogical research on the families of all inhabitants with confirmed parkinsonism and compiled the pedigrees. These were modified on the basis of information from a consecutive door-to-door survey and local municipal and church registers. Results: In the first stage, three large pedigrees with a familial occurrence of parkinsonism were found; two originated in one of the region's villages. In the second stage, these two pedigrees were combined into one large family tree. Conclusions: The high prevalence of parkinsonism in the researched area is caused by the familial aggregation of parkinsonism that was found in two large family trees. This is probably the result of the genetic isolation of the regional population due to the very low migration rate of its inhabitants to neighboring regions in the last two centuries.
first_indexed 2024-12-13T11:40:46Z
format Article
id doaj.art-18bc6b39476c45b2acc8b29d79471ca6
institution Directory Open Access Journal
issn 1213-8118
1804-7521
language English
last_indexed 2024-12-13T11:40:46Z
publishDate 2016-03-01
publisher Palacký University Olomouc, Faculty of Medicine and Dentistry
record_format Article
series Biomedical Papers
spelling doaj.art-18bc6b39476c45b2acc8b29d79471ca62022-12-21T23:47:39ZengPalacký University Olomouc, Faculty of Medicine and DentistryBiomedical Papers1213-81181804-75212016-03-01160115816010.5507/bp.2015.013bio-201601-0023Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech RepublicKaterina Mensikova0Marek Godava1Petr Kanovsky2Pavel Otruba3Michaela Kaiserova4Miroslav Vastik5Lenka Mikulicova6Tereza Bartonikova7Radek Vrtel8Radek Vodicka9Sandra Kurcova10Petr Jugas11Josef Ovecka12Ludmila Sachova13Frantisek Dvorsky14Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicDepartment of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech RepublicNeurology Outpatient Clinic, Veseli nad Moravou, Czech RepublicGeneral Practitioner, Lipov, Czech RepublicGeneral Practicioner, Velka nad Velickou-Javornik, Czech RepublicGeneral Practicioner, Velka nad Velickou-Javornik, Czech RepublicBackground: An epidemiological study conducted over four years revealed increased prevalence of neurodegenerative parkinsonism in a small, isolated region (10 villages, with a combined population of 8664, with approx. 2927 over 50 years of age) of south-eastern Moravia, Czech Republic. The aim of this study was to obtain more detailed information on the medical history of the relatives of individuals with confirmed parkinsonism in an isolated rural population in south-eastern Moravia, Czech Republic. Methods: We did detailed genealogical research on the families of all inhabitants with confirmed parkinsonism and compiled the pedigrees. These were modified on the basis of information from a consecutive door-to-door survey and local municipal and church registers. Results: In the first stage, three large pedigrees with a familial occurrence of parkinsonism were found; two originated in one of the region's villages. In the second stage, these two pedigrees were combined into one large family tree. Conclusions: The high prevalence of parkinsonism in the researched area is caused by the familial aggregation of parkinsonism that was found in two large family trees. This is probably the result of the genetic isolation of the regional population due to the very low migration rate of its inhabitants to neighboring regions in the last two centuries.https://biomed.papers.upol.cz/artkey/bio-201601-0023_Familial_autosomal-dominant_neurodegenerative_parkinsonism_with_cognitive_deterioration_spanning_five_generati.phpparkinsonismcognitive deteriorationisolated populationneuroepidemiologygenealogy
spellingShingle Katerina Mensikova
Marek Godava
Petr Kanovsky
Pavel Otruba
Michaela Kaiserova
Miroslav Vastik
Lenka Mikulicova
Tereza Bartonikova
Radek Vrtel
Radek Vodicka
Sandra Kurcova
Petr Jugas
Josef Ovecka
Ludmila Sachova
Frantisek Dvorsky
Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
Biomedical Papers
parkinsonism
cognitive deterioration
isolated population
neuroepidemiology
genealogy
title Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
title_full Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
title_fullStr Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
title_full_unstemmed Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
title_short Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic
title_sort familial autosomal dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south eastern moravia czech republic
topic parkinsonism
cognitive deterioration
isolated population
neuroepidemiology
genealogy
url https://biomed.papers.upol.cz/artkey/bio-201601-0023_Familial_autosomal-dominant_neurodegenerative_parkinsonism_with_cognitive_deterioration_spanning_five_generati.php
work_keys_str_mv AT katerinamensikova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT marekgodava familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT petrkanovsky familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT pavelotruba familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT michaelakaiserova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT miroslavvastik familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT lenkamikulicova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT terezabartonikova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT radekvrtel familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT radekvodicka familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT sandrakurcova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT petrjugas familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT josefovecka familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT ludmilasachova familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic
AT frantisekdvorsky familialautosomaldominantneurodegenerativeparkinsonismwithcognitivedeteriorationspanningfivegenerationsinageneticallyisolatedpopulationofsoutheasternmoraviaczechrepublic