Abstract
The Kii peninsula of Japan and Guam are the highest incidence foci of ALS in the world.1 We reported the continuous high prevalence of ALS and the concomitant occurrence of many parkinsonism–dementia complex (PDC) patients in the Kii peninsula (Kii ALS/PDC), including the first neuropathologically verified cases.2,3⇓ This contrasts the sharp decline of the incidence rates of ALS/PDC on Guam after 1970. In 1989, the high prevalence of peculiar pigmentary retinopathy (PR) resembling ophthalmomyiasis interna posterior (OIP) was reported in the Guamanian Chamorro population.4 OIP-like PR shows linear and crisscrossed patterns of retinal pigment epithelial disruption and was named “pigmentary retinopathy of Guam” (PROG). PROG was suspected to be etiologically associated with ALS/PDC or one of the clinical features of ALS/PDC because it was frequently seen in individuals with ALS/PDC. To explore whether PR occurs in patients with Kii ALS/PDC, we performed ophthalmoscopy on five patients and found PR resembling PROG in a PDC patient. One …
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