Neuropediatrics 2012; 43 - PS14_06
DOI: 10.1055/s-0032-1307104

Hypocretin-level during therapy with intravenous immunglobulines in an early stage of narcolepsy with cataplexy in childhood

M Prüfer 1, J Schönfelder 1, D Friebel 1, V Mall 1, M von der Hagen 1, M Smitka 1
  • 1Abteilung Neuropädiatrie, Universitätsklinikum Carl Gustav Carus, Dresden, Germany

Aims: Narcolepsy is a chronic sleep disorder of central origin associated with hypersomnia, cataplexy and specific changes in REM-sleep. A reduced CSF-(cerebrospinal fluid)-level of hypocretin is a typical finding in patients with narcolepsy. Hypocretin is a neuropeptide which is secreted by a certain group of neurons in the hypothalamus and regulates the sleep-wake rhythm. Autoimmunological processes are assumed to be responsible for the destruction of hypocretin secreting neurons in hypothalamus. The association with HLA-DQB1*0602 points to a possible genetic cause. In single patients intravenous immunglobulines (IVIG) displayed a consistent positive effect on the narcoleptic symptoms. In order to estimate the effect of IVIG-therapy upon hypocretin-levels, hypocretin was determined before and after IVIG-therapy.

Methods: Our patient experienced at an age of 8 years and 3 months increased daytime sleepiness, cataplectic episodes and hypnagogic hallucinations. Ullanlinna-Narcolepsy-Scale and Epworth-Sleepiness-Scale showed urgent indications for the presence of narcolepsy. The pathologically reduced sleep onset periods in multi-sleep-latency-test corroborated the assumed diagnosis. The patient was positive for HLA-DQB1*0602.

Results: At diagnosis the CSF hypocretin level was significantly reduced (51 pg/ml). The patient was treated with methylphenidate and IVIG. IVIG was given in a dose of 2mg/kg body weight every 4 weeks in a total of 5 cycles. After finishing therapy the CSF hypocretin level was determined again and was unchanged (50 pg/ml).

Conclusion: A correlation between the improvement of clinical signs after IVIG-therapy and CSF hypocretin level could not be found. In the literature there was no increase of CSF hypocretin level immediately after completion of IVIG-therapy in most cases. Further clinical investigations could show if there is a long-term correlation between sustained improvement of clinical signs and normalization of CSF hypocretin levels.

hypocretin - narcolepsy - intravenous immunglobulines (IVIG)