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Kleine-Levin-Syndrome in Pregnancy:
A Case Report
S. von Manitius1, D. Flugel1, M. Tafti2, B. Tettenborn1
1KSSG, Neurology, St. Gallen, Switzerland, 2Center for
Investigation and Research in Sleep , Lausanne, Switzerland
Background and aims: Kleine-Levin syndrome (KLS) is a
rare sleep disorder, predominantly affecting males during
adolescence. We report a female patient with recurrent
episodes of hypersomnia and a long sleeping episode during
pregnancy.
Methods: A 26-years-old female patient is presented with
recurrent episodes of hypersomnia, starting at the age of 12,
shortly before menarche.
Results: Sleep episodes occurred in a frequency of 2-3/year
with varying duration, the longest being 3 weeks in 2008,
when the patient presented at our clinic the first time. In
addition, altered perception and cognitive dysfunction were
present, but no hyperphagia or hypersexuality, fulfilling the
criteria of the international classification for sleep disorders.
Polysomnography, actigraphy and MSLT showed normal
results during asymptomatic phases. Encephalopathy was
excluded by laboratory results, normal cMRI and normal
hypocretin-1 level in cerebrospinal fluid.
After 2010, the episodes became less frequent and shorter
until 2017, when the patient developed hypersomnia lasting
from the 8th to the 18th week of pregnancy.
Polysomnography showed a sleep duration of >14 hours
with normal sleep architecture, 8 sleep cycles. Genetics
confirmed HLA positivity for HLA DQB1*0201 with
maternal transmission. For 2 weeks the patient was treated
with i.v. infusions and s.c. heparin to prevent dehydration
and thrombosis. The patient slowly became more vigilant
and mobile without using any medication because of
pregnancy.
Conclusion: This is the first case of KLS with a dramatic
hypersomnia episode during pregnancy. Sleep studies and
HLA-results are presented and discussed together with
pathophysiology.