HIGH PREVALENCE OF KENNEDYS-DISEASE IN WESTERN FINLAND - IS THE SYNDROME UNDERDIAGNOSED

Citation
B. Udd et al., HIGH PREVALENCE OF KENNEDYS-DISEASE IN WESTERN FINLAND - IS THE SYNDROME UNDERDIAGNOSED, Acta neurologica Scandinavica, 98(2), 1998, pp. 128-133
Citations number
20
Categorie Soggetti
Clinical Neurology
ISSN journal
00016314
Volume
98
Issue
2
Year of publication
1998
Pages
128 - 133
Database
ISI
SICI code
0001-6314(1998)98:2<128:HPOKIW>2.0.ZU;2-1
Abstract
Objectives - To assess the prevalence of Kennedy's disease in the Vasa region of Western Finland. Patients and methods - Verification of dia gnosis by molecular genetic techniques since 1995. Results - Within 2 years we have been able to identify a large number of families with th is disorder. We have arrived at a point prevalence of Kennedy's diseas e in the district of Vasa Central Hospital of 13 patients per 85,000 m ale inhabitants. Assuming an even distribution throughout the country, this would suggest hundreds of patients with this disorder in Finland . Conclusion - Kennedy's disease is the most common motor neuron disor der in the Vasa region, exceeding the prevalence of ALS by a factor of two, and far more common than any of the other motor neuron disorders . The fact that none of our patients, despite previous examinations, h ad correct diagnoses before 1995, indicates that Kennedy's disease sti ll might be relatively underdiagnosed.