The term 'endemic parkinsonism' refers to diseases that manifest with a dominant parkinsonian syndrome, which can be typical or atypical, and are present only in a particular geographically defined location or population. Ten phenotypes of endemic parkinsonism are currently known: three in the Western Pacific region; two in the Asian-Oceanic region; one in the Caribbean islands of Guadeloupe and Martinique; and four in Europe. Some of these disease entities seem to be disappearing over time and therefore are probably triggered by unique environmental factors. By contrast, other types persist because they are exclusively genetically determined. Given the geographical clustering and potential overlap in biological and clinical features of these exceptionally interesting diseases, this Review provides a historical reference text and offers current perspectives on each of the 10 phenotypes of endemic parkinsonism. Knowledge obtained from the study of these disease entities supports the hypothesis that both genetic and environmental factors contribute to the development of neurodegenerative diseases, not only in endemic parkinsonism but also in general. At the same time, this understanding suggests useful directions for further research in this area.

1st Department of Neurology Masaryk University Medical School Brno Czech Republic

Département de Neuropathologie Hôpital La Pitié Salpêtrière Paris France

Départment de Neurologie Centre Hospitalier Universitaire de la Guadeloupe Pointe á Pitre France

Department of Clinical and Molecular Genetics Faculty of Medicine and Dentistry Palacky University Olomouc Czech Republic

Department of Clinical and Molecular Pathology Faculty of Medicine and Dentistry Palacky University Olomouc Czech Republic

Department of Human Neurophysiology Fukushima Medical University Fukushima Japan

Department of Neurology and Clinical Neuroscience Center Faculty of Medicine and Dentistry Palacky University Olomouc Czech Republic

Department of Neurology Faculty of Medicine Johannes Kepler University Linz Austria

Department of Neurology Hospital General Universitario Gregorio Marañón Madrid Spain

Department of Neurology Kuwana City Medical Center Kuwana Japan

Department of Neurology Santa Maria University Hospital Terni Italy

Department of Neurology School of Medicine Oregon Institute of Occupational Health Sciences Oregon Health and Science University Portland OR USA

Department of Pathology 3rd Medical Faculty Charles University and University Hospital Kralovske Vinohrady Prague Czech Republic

Department of Pathology and Molecular Medicine 3rd Medical Faculty Charles University and Thomayer University Hospital Prague Czech Republic

Independent Researcher Bali Indonesia

Laboratory of Growth Regulators Faculty of Science Palacky University Olomouc Czech Republic

Lille Neuroscience and Cognition Research Centre INSERM U1172 Lille France

Research Center for Health Sciences Faculty of Medicine and Surgery University of Santo Tomás Manila The Philippines

St Anne University Hospital Brno Czech Republic

St Luke's Institute of Neuroscience Metro Manila The Philippines

University Hospital Olomouc Czech Republic

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