ABSTRACT
The extremely close association of HLA DR2 to narcolepsy with cataplexy was first noted in 1984 (1). Later work showed that the disorder was actually associated with HLA DQB10602 (2) and that the HLA gene itself, rather than another in close linkage disequilibrium, was the true marker of the disorder (3). Most cases of human narcolepsy are sporadic and monozygotic twins are usually discordant for the disease (4). Narcolepsy tends to start relatively abruptly, most often in the 2nd decade of life (5). These considerations have resulted in the formulation of an autoimmune hypothesis to explain the pathogenesis of human sporadic narcolepsy. This paper will summarize the available data.
