The first description of Chiari I malformation with intuitive correlation between tonsillar ectopia and syringomyelia

Abstract

Few are familiar with the neurological contributions of the German pathologist Theodor Langhans. Even fewer are aware of his significant and early contributions to the study of what is now known as the Chiari I malformation. In at least 4 cases, Langhans described the association between tonsillar ectopia and syringomyelia. Moreover, this early pioneer speculated that there was a cause and effect with hindbrain herniation resulting in improper flow at the craniocervical junction and consequent development of syringomyelia. These cases were reported prior to Hans Chiari's descriptions, and Langhans' theory of impeded foramen magnum flow as a cause of syringomyelia was novel and preceded the current understanding of this mechanism by almost a century. The authors discuss the life of Langhans and translate excerpts from his 1881 work regarding tonsillar ectopia and syringomyelia.