title: [A description of early hydrocephalus with macrocephaly, cerebral palsy, epilepsy, intellectual disability, and growth retardation by Norwegian novelist Amalie Skram (1846-1905)].
abstract: In her novel «S.G. Myre» written in 1890, Norwegian author Amalie Skram describes Hans (Hansemann) Tønnesen, a 16-year-old boy with an enormous head, convulsions, and a rudimentary command of language. He is bullied by children in the street and is held in low esteem by his mother, who refers to him as a beast, crazy, or «a cross». Presumably, the boy has hydrocephalus with macrocephaly, epilepsy, and mental retardation. His retarded growth, small teeth and cracked skin suggest growth hormone deficiency resulting from his hydrocephalus. A couple of interesting details, such as his «splaying his fingers and moving them in various directions to make himself understood» and his «becoming red with exertion and grimacing terribly» when trying to speak, suggest extrapyramidal, dyskinetic cerebral palsy. He may also be deaf. A cousin of Skram's shared several of these traits and is a likely model for Hansemann. A question that occurs to a modern reader is to what degree Hansemann is mentally retarded, or whether he is wrongly perceived as such because of his physical challenges and his communication problems.
authors: Avdeling for nevrohabilitering, Oslo universitetssykehus, Ullevål, Norway. bjornar.hassel@ffi.no
authors: Bjørnar
authors: B
authors: Hassel
mesh_terms: D000293:Adolescent; D004827:Epilepsy / Q000266:history*; D049672:History, 19th Century; D006801:Humans; D006849:Hydrocephalus / Q000266:history*; D008607:Intellectual Disability / Q000266:history*; D008093:Literature, Modern / Q000266:history; D008297:Male; D008513:Medicine in Literature; D058627:Megalencephaly / Q000266:history*; D009664:Norway