Review elements of evidence supporting 17^th century description of endemic ALS in Kii peninsula reported by Yoshino Yase in Kuzuhara 2021.

Kumano Province (Muro County of Kii) was regarded as the Holy Land of Japanese Shintoism since the 7th century containing three Grand Shrines of Japanese Shintoism (Taisha). Yoshino Yase identified a book from the 17^th century titled “Honcho Koji Innen Shu” that was a collection (“Shu”) of tales and legends in the past (“Koji”) which had been handed down by oral history in Japan (“Honcho”).  Each story is accompanied by a coda “Innen” - the karma in Buddhism that describes the consequence resulting from the reported event. The book is woodblock-printed with Chinese ink on Japanese paper in kanji (Chinese character) containing 156 short mysterious stories and legends collected in the 17th century from all over Japan. Each story usually consists of two parts: first, a presentation of real-looking but mysterious events, and second, comments of the writer and explanation by the karma (its effect and cause) to be learned from the story.

The 92nd story expounds the clinical report that Kinbei Yamamoto developed, without fever, motor paraplegia of subacute onset and of chronic course without remission or recovery named “paraplegia of Koza.” Further description included endemic paraplegia among inhabitants of Koza Village for generations” resembling   endemic ALS in the Kozagawa focus for generations.

Elements of geography, culture and history support that “mysterious paraplegia of Koza” existed as the same disorder similar to Kii ALS of the hyperendemic focus of Kozagawa at present.

No visual depiction of the muscle mass in reported cases are presented.  The slow progression and repeated occurrence in later generations increases the likelihood that the reported cases may have been present for two centuries prior to the first modern reports.