title: [Diagnostic Criteria for Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex in the Kii Peninsula, Japan].
abstract: The diagnostic criteria for amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula of Japan (Kii ALS/PDC) have been proposed. Muro disease has been considered an endemic neurodegenerative disease in the southern part of the Kii peninsula. Recent intensive and comprehensive research has revealed it to be a complex and genetically heterogeneous disease. At present, there are four subtypes of Muro disease: sporadic amyotrophic lateral sclerosis (ALS), Kii ALS/PDC with tauopathy, ALS with C9orf72 gene mutation, and ALS with optineurin gene mutation. The present criteria are applicable to only one of these subtypes, Kii ALS/PDC with tauopathy, which is quite similar to ALS/PDC in Guam.
authors.affiliation: Kii ALS/PDC Research Center, Graduate School of Regional Innovation Studies, Mie University.
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Kokubo
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D003704:Dementia / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D020734:Parkinsonian Disorders / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D012720:Severity of Illness Index