title: [An account of a strange instance of disease--Stengel-Batten-Spielmayer-Vogt disease].
abstract: Otto Christian Stengel's (1794-1890) description of four siblings suffering from probable neuronal ceroid lipofuscinosis (Batten disease or Spielmeyer-Vogt disease) is one of the few Norwegian primary reports of neurological disease. Dr. Stengel was a physician in the copper mining town of Roeros in Norway from 1821 to 1883. His report was published in Norwegian in the first volume of the first Norwegian medical journal "Eyr" in 1826, 77 years before Frederick E. Batten (1865-1918) through his publication in 1903 became internationally renowned for giving the first report of the disease. This article presents the life of Dr. Stengel and reviews the findings in his report.
authors.affiliation: Nevrologisk avdeling, Sykehuset i Vestfold, Tønsberg, Halvdan Wilhelmsens allée 16, 3116 Tønsberg. are.brean@siv.no
authors.forename: Are
authors.initials: A
authors.lastname: Brean
mesh_terms: D049671:History, 18th Century; D049672:History, 19th Century; D006801:Humans; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics / Q000266:history*; D009664:Norway