Antoine Léotard1, Elisabeth Groos1, Charlotte Chaumereuil1, Laure Peter-Derex1, Andrea O Rossetti1, Smaranda Leu-Semenescu1, Isabelle Arnulf2. 1. From the Sleep Disorders Unit (A.L., E.G., C.C., S.L.-S., I.A.), National Reference Center for Narcolepsy, Hypersomnia and Kleine-Levin Syndrome, Brain Research Institute (CRICM, UPMC, Paris 6; Inserm UMR_S 975; CNRS UMR 7225), Sorbonne University Paris; Sleep Medicine and Respiratory Disease Center (L.P.-D.), Competence Center for Narcolepsy, Hypersomnia and Kleine-Levin Syndrome, Hospices Civils de Lyon, France; and Center for Investigation and Research in Sleep (A.O.R.), Lausanne University Hospital and Lausanne University, Switzerland. 2. From the Sleep Disorders Unit (A.L., E.G., C.C., S.L.-S., I.A.), National Reference Center for Narcolepsy, Hypersomnia and Kleine-Levin Syndrome, Brain Research Institute (CRICM, UPMC, Paris 6; Inserm UMR_S 975; CNRS UMR 7225), Sorbonne University Paris; Sleep Medicine and Respiratory Disease Center (L.P.-D.), Competence Center for Narcolepsy, Hypersomnia and Kleine-Levin Syndrome, Hospices Civils de Lyon, France; and Center for Investigation and Research in Sleep (A.O.R.), Lausanne University Hospital and Lausanne University, Switzerland. isabelle.arnulf@aphp.fr.
Abstract
OBJECTIVE: To retrospectively compare the benefits (episode cessation) and risks of IV methylprednisolone (IV-MP) vs abstention during prolonged Kleine-Levin syndrome (KLS) episodes. METHODS: A total of 26 patients with KLS received 1 g/d IV-MP for 3 days during 1 to 6 episodes each (totaling 43 IV-MP sessions). The change of episode duration with IV-MP (vs previous episode duration) was compared with the change duration between 2 consecutive episodes in 48 untreated patients matched for age, sex, age at KLS onset, number of episodes, and disease duration (more treated than untreated patients had long episodes). RESULTS: Eleven patients (42.3%) had an episode that was at least 1 week shorter than the preceding one when they received IV-MP therapy, whereas shorter episodes were significantly less frequent (10.4%) in the untreated group. This benefit was more marked (65.5% responders, 12 fewer days in an episode vs 0 days in the untreated patients) when IV-MP was infused before the 10th day of the episode. Mild, transient adverse effects (insomnia, muscle pain, nervousness/restlessness, but no manic switching) were reported by 61.3% of patients. No specific responder profile was identified. CONCLUSION: In this open-labeled, naturalistic study, early IV-MP (following the protocol for multiple sclerosis relapses) had a good benefit/risk ratio during KLS episodes in patients with long episodes (with half of the patients having an early cessation of episodes). CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with long episodes of KLS, IV steroids decrease the duration of KLS episodes.
OBJECTIVE: To retrospectively compare the benefits (episode cessation) and risks of IV methylprednisolone (IV-MP) vs abstention during prolonged Kleine-Levin syndrome (KLS) episodes. METHODS: A total of 26 patients with KLS received 1 g/d IV-MP for 3 days during 1 to 6 episodes each (totaling 43 IV-MP sessions). The change of episode duration with IV-MP (vs previous episode duration) was compared with the change duration between 2 consecutive episodes in 48 untreated patients matched for age, sex, age at KLS onset, number of episodes, and disease duration (more treated than untreated patients had long episodes). RESULTS: Eleven patients (42.3%) had an episode that was at least 1 week shorter than the preceding one when they received IV-MP therapy, whereas shorter episodes were significantly less frequent (10.4%) in the untreated group. This benefit was more marked (65.5% responders, 12 fewer days in an episode vs 0 days in the untreated patients) when IV-MP was infused before the 10th day of the episode. Mild, transient adverse effects (insomnia, muscle pain, nervousness/restlessness, but no manic switching) were reported by 61.3% of patients. No specific responder profile was identified. CONCLUSION: In this open-labeled, naturalistic study, early IV-MP (following the protocol for multiple sclerosis relapses) had a good benefit/risk ratio during KLS episodes in patients with long episodes (with half of the patients having an early cessation of episodes). CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with long episodes of KLS, IV steroids decrease the duration of KLS episodes.
Authors: Kiran Maski; Lynn Marie Trotti; Suresh Kotagal; R Robert Auger; Todd J Swick; James A Rowley; Sarah D Hashmi; Nathaniel F Watson Journal: J Clin Sleep Med Date: 2021-09-01 Impact factor: 4.324