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IV steroids during long episodes of Kleine-Levin syndrome
Antoine Léotard1, Elisabeth Groos1, Charlotte Chaumereuil1
1From 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.
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.
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