PMID- 30410634 OWN - NLM STAT- MEDLINE DCOM- 20190415 LR - 20190415 IS - 1875-8584 (Electronic) IS - 0953-4180 (Print) IS - 0953-4180 (Linking) VI - 2018 DP - 2018 TI - Intravenous Immunoglobulin Therapy Administered Early after Narcolepsy Type 1 Onset in Three Patients Evaluated by Clinical and Polysomnographic Follow-Up. PG - 1671072 LID - 10.1155/2018/1671072 [doi] LID - 1671072 AB - Narcolepsy type 1 is a rare disabling sleep disorder mainly characterized by excessive daytime sleepiness and cataplexy, an emotion-triggered sudden loss of muscle tone. Patients have a selective degeneration of hypocretin-producing neurons in the dorsolateral posterior hypothalamus with growing evidence supporting the hypothesis of an autoimmune mechanism. Few case studies that reported intravenous immunoglobulin therapy (IVIg) suggest the efficacy of IVIg when administered early after disease onset, but the results are controversial. In these retrospective case observations, IVIg cycles were initiated within one to four months after cataplexy onset in a twenty-seven-year-old man, a ten-year-old girl, and a seven-year-old boy, all three with early onset typical narcolepsy type 1. Efficacy of treatment (three IVIg cycles of 1 g/kg administered at four-week intervals) was evaluated based on clinical, polysomnographic, and multiple sleep latency test (mean latency and SOREM) follow-up. Two patients reported decreased cataplexy frequency and ameliorated daytime sleepiness, but no significant amelioration of polysomnographic parameters was observed. Given the possibility of spontaneous improvement of cataplexy frequency with self-behavioral adjustments, these observations would need to be confirmed by larger controlled studies. Based on the present study and current literature, proof of concept is still missing thus prohibiting the consideration of IVIg as an efficient treatment option. FAU - Ruppert, Elisabeth AU - Ruppert E AUID- ORCID: 0000-0002-0361-0985 AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. FAU - Zagala, Helene AU - Zagala H AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. FAU - Chambe, Juliette AU - Chambe J AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. AD - Department of General Practice, School of Medicine, 4 rue Kirschleger, 67085 Strasbourg, France. FAU - Comtet, Henri AU - Comtet H AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. FAU - Kilic-Huck, Ulker AU - Kilic-Huck U AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. FAU - Lefebvre, Francois AU - Lefebvre F AD - Biostatistics Department, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. FAU - Bataillard, Marc AU - Bataillard M AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. FAU - Schroder, Carmen AU - Schroder C AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. AD - Department of Child Psychiatry, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. FAU - Calvel, Laurent AU - Calvel L AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. AD - Department of Palliative Care, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. FAU - Bourgin, Patrice AU - Bourgin P AD - Department of Neurology, Sleep Disorders Center, CIRCSom, Hopital Civil, 1 place de l'Hopital, 67091 Strasbourg, France. AD - Institute for Cellular and Integrative Neurosciences, CNRS-UPR 3212, University of Strasbourg, 5 rue Blaise Pascal, 67000 Strasbourg, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20181015 PL - Netherlands TA - Behav Neurol JT - Behavioural neurology JID - 8914585 RN - 0 (Immunoglobulins, Intravenous) RN - Narcolepsy 1 SB - IM MH - Adult MH - Child MH - Female MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage/*pharmacology MH - Male MH - Narcolepsy/*drug therapy/physiopathology MH - Polysomnography PMC - PMC6205306 EDAT- 2018/11/10 06:00 MHDA- 2019/04/16 06:00 PMCR- 2018/10/15 CRDT- 2018/11/10 06:00 PHST- 2018/03/14 00:00 [received] PHST- 2018/08/26 00:00 [accepted] PHST- 2018/11/10 06:00 [entrez] PHST- 2018/11/10 06:00 [pubmed] PHST- 2019/04/16 06:00 [medline] PHST- 2018/10/15 00:00 [pmc-release] AID - 10.1155/2018/1671072 [doi] PST - epublish SO - Behav Neurol. 2018 Oct 15;2018:1671072. doi: 10.1155/2018/1671072. eCollection 2018.