General Child Neurology
Autosomal dominant hereditary ataxias
May. 17, 2022
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This woman has primary generalized dystonia manifested by dystonic camptocormia and “dromedary” gait. The term "camptocormia" (literally, bent tree trunk) is believed to have been coined in 1914 by Alexandre Achille Souques, a student of Charcot and leading French neurologist of his day. Camptocormia, first reported in soldiers in World War I and believed to represent a psychogenic disorder, is now recognized as a form of axial dystonia seen in patients with isolated dystonia or in patients with Parkinson disease and other movement disorders (Azher and Jankovic 2005; Jankovic 2010). It now has an extensive differential diagnosis. "Dromedary" refers to the desert-dwelling, single-humped camel, which avoids trotting or galloping to conserve energy, instead ambling with a peculiar rolling gait (like the rolling of a boat on rough waters) that results from its lifting both forelegs and hind legs on 1 side together. The term may have been first used in neurology by Hermann Oppenheim, the acknowledged leader of German neurology, at the end of the 19th century. (Contributed by Dr. Joseph Jankovic.)