Alice Vance (?Das B�renweib?): A historical case of Nievergelt syndrome

Abstract

Several malformed individuals were presented at the World Exhibition in Antwerp in 1894. Among them was Mrs. Alice Vance from Mount Pleasant, Texas, with congenital limb defects, and Mr. Eugen Berry, who had asymmetrical, monstrous enlargement and macrodactyly of the feet, i.e., Proteus syndrome. After the World Exhibition Mrs. Vance presented herself to the public in Castan's Panopticon imitating a bear. She became famous under the stage name "Das Bärenweib" ("the bear-like woman") and was examined by several German clinicians, and her malformations were considered to be of high scientific interest. Mrs. Vance had mesomelic dwarfism and her mother was known to have similar malformations. Her limb deficiencies were generally considered a unique congenital condition those days, and the diagnosis of "a maternally inherited malformation of the forearms and the shanks" [Daffner 1898: Munch Med Wochenschr 25:782] was made. Virchow [1897: Verh Berl Ges Ethnol Urgeschichte 29:624], feeling attacked by a daily newspaper stating that the physicians as well as the police of Berlin had missed the diagnosis of an "English disease," eventually exercised his authority and diagnosed Alice Vance as a "phocomelic." Clearly, she was not a phocomelic according to past and current definition of this term. Thus, from a historical point of view, the story illustrates how pressure from the daily press altered the definition of an up-to-then precisely defined medical term for decades. According to the clinical data and an X-ray report available from the literature, Alice Vance had a dominantly inherited type of mesomelic dwarfism. We propose the diagnosis of Nievergelt syndrome.

Patient with the mesomelic dysplasia, Nievergelt syndrome, and cerebellovermian agenesis and cataracts

“…Salonen and Sulaman [1958] described shortening and rhomboid or triangular deformity of tibiae and radii on x‐ray films, which are specific features of the syndrome. There are a few further sporadic reports on patients with NS [Young and Wood, 1974; Burnstein et al, 1989; Petrella et al, 1990; Urban and Kruger, 1998].…”