ABSTRACT

The prevalence of narcolepsy-cataplexy is, according to the latest multicentric epidemiological studies (1,2), approximately 0.05 in European as well as in North American population. Although most of the patients have had the symptoms of narcolepsy since youth, a large prospective series identified only 5% of the cases as prepubertal (3). The most current pathophysiologic model for narcolepsy involving an autoimmunemediated destruction of hypocretin (Hcrt)/orexin-containing neurons (4) suggests the importance of early diagnosis of the disease. Some recent studies (5,6) have shown a favourable effect of autoimmune suppressive treatment using intravenous immunoglobulins in early stages of the disease, but they were open labeled trials and all positive effects were reported subjectively. Other authors have not confirmed this experience with steroid treatment (7).