Immunogenetics of Narcolepsy

The important role of genetic factors in the pathogenesis of narcolepsy was realized very early in the course of research on this disease. One of the first recognized instances of narcolepsy, reported by Westphal in 1877 (1), was a familial case, as the mother of his patient suffered from the same disease. More than 100 years later, in 1984, the groups of Juji and Honda in Tokyo (2), and of Langdon and Parkes in London (3), found what could be the biochemical basis of hereditary factors in narcolepsy. Almost simultaneously they reported that all of their narcoleptic patients carry the HLA type DR2, while this antigen is present in only 25—33% of the general population. Since then, immunogenetic methods have played a crucial role in narcolepsy research. As this field may not be familiar to those working in traditional areas of sleep research, this presentation will first give a short introduction, and then summarize some recent immunogenetic data which further outline the role of the HLA-system in the pathogenesis of narcolepsy.