| dc.description.abstract | Background: Narcolepsy is a chronical neurological disease with a prevalence of 0,02-0,05 % in the western world. A massive increase in incidence occurred after the vaccination with Pandemrix against the H1N1-influenza. The clinical picture is characterized by excessive daytime sleepiness (EDS) with irresistible sleep attacks, cataplexy, sleep paralysis, sleep related hallucinations and nocturnal sleep disturbances. Available treatment is symptomatic and, although relieving, often inadequate in alleviating the plethora of associated symptoms. Sodium Oxybate (SXB) has in several studies shown a substantial effect in reduction of sleep related symptoms as well as cataplexy, thus presenting it as a versatile alternative to traditional combination treatment with stimulants and antidepressants. Efficacy and side effects are, however, sparsely examined in the pediatric population.
Aim: To investigate the effect of SXB on EDS and cataplexy in a population of patients with narcolepsy and to explore what side effects the population under examination has reported.
Methods: This retrospective study was given a population based design with the aim of including all patients with narcolepsy diagnosed according to ICSD-2.2 during 2000–2010 in the county of Halland and Västra Götaland treated with sodium oxybate. The evaluation of EDS was based on patient assessments with the Epworth Sleepiness Scale (ESS) and the evaluation of cataplexy on assessments made with the Stanford Cataplexy Scale. Evaluation of the substance under investigation was based on a comparison of assessments executed before and after initiated treatment with SXB registered in “The National Swedish Register on Narcolepsy”. The evaluation of side effects was based on data from medical records.
Results: Eighteen patients were identified and all but one had Pandemrix related narcolepsy. The median age was 18 years (range 11–22) of which seven were women. The median ESS scale score was decreased from 18, at the last evaluation before treatment, to 13.5, on the first assessment after three months of treatment. The median cataplexy scale score, compared using the same criteria, decreased from 10 to 8.5. Side effects were reported by 44 % of the patients, the most prevalent being gastrointestinal symtoms, decreased appetite, headache and perspiration. No patient abolished the treatment due to side effects.
Conclusions: In this population based study a significant decrease of EDS and a non- significant decrease of cataplexy was demonstrated after commenced treatment with SXB. The method of measuring cataplexy, practiced in this study, differs from the majority of similar studies, and its ability to detect all nuances of clinical relevance has in this study proved to be limited. The majority of the patients tolerated the substance well. This is the first study to conduct research based on data from the National Swedish Register on Narcolepsy.
Keywords: narcolepsy, children, young adults, sodium oxybate, cataplexy, excessive daytime sleepiness, side effects | sv |
