Neurológia pre prax 5/2024

Progressive supranuclear palsy and Guam parkinsonian complex or a Canadian in the Pacific – John C. Steele (1934–2022)“

John Charles Steele (1934–2022) was a Canadian neurologist who, together with Richardson and Olszewski, described a new nosological entity that was originally named eponymously; however, over time, it became known as progressive supranuclear palsy. Later, he was concerned with epidemiological and clinical research on the endemic disease of amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam, documenting its occurrence, phenotypes, clinical manifestations, heredity, and decreasing incidence as well as prevalence. He also studied the pathophysiology of this disease, for years holding the view that it was a hereditary polygenic disease; it was only at the end of his life that he accepted the hypothesis which attributed the manifestation of the disease to chronic intoxication with neurotoxic compounds contained in cycad fruits: beta-methylamino-L-alanine (BMAA) and methylazoxymethanol (MAM).

Keywords: progressive supranuclear paralysis, parkinsonism-dementia complex of Guam