title: Familial, autosomal-dominant neurodegenerative parkinsonism with cognitive deterioration spanning five generations in a genetically isolated population of south-eastern Moravia, Czech Republic.
abstract: 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.
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Katerina
authors: K
authors: Mensikova
authors: Department of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Marek
authors: M
authors: Godava
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Petr
authors: P
authors: Kanovsky
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Pavel
authors: P
authors: Otruba
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Michaela
authors: M
authors: Kaiserova
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Miroslav
authors: M
authors: Vastik
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Lenka
authors: L
authors: Mikulicova
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Tereza
authors: T
authors: Bartonikova
authors: Department of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Radek
authors: R
authors: Vrtel
authors: Department of Medical Genetics and Fetal Medicine, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Radek
authors: R
authors: Vodicka
authors: Department of Neurology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
authors: Sandra
authors: S
authors: Kurcova
authors: Neurology Outpatient Clinic, Veseli nad Moravou, Czech Republic.
authors: Petr
authors: P
authors: Jugas
authors: General Practitioner, Lipov, Czech Republic.
authors: Josef
authors: J
authors: Ovecka
authors: General Practicioner, Velka nad Velickou-Javornik, Czech Republic.
authors: Ludmila
authors: L
authors: Sachova
authors: General Practicioner, Velka nad Velickou-Javornik, Czech Republic.
authors: Frantisek
authors: F
authors: Dvorsky
mesh_terms: D000368:Aged; D003072:Cognition Disorders / Q000453:epidemiology* / Q000235:genetics; D018153:Czech Republic / Q000453:epidemiology; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020734:Parkinsonian Disorders / Q000453:epidemiology* / Q000235:genetics; D010375:Pedigree
keywords: cognitive deterioration; genealogy; isolated population; neuroepidemiology; parkinsonism