SARS-CoV-2 Infection Causes Relapse of Kleine-Levin Syndrome: Case Report and Review of Literature.
Marčić Marino,Marčić Ljiljana,Marčić Barbara
Neurology international
Recurrent episodes of hypersomnia, hypersexuality, compulsive eating, behavioral and cognitive disturbances, are the basic clinical features of Kleine-Levin syndrome (KLS). Our case report describes a patient who was diagnosed with KLS at the age of 20. With appropriate therapy, the disease had a satisfactory course until patient had a moderate form of SARS-CoV-2 infection, which led to a significant exacerbation of all symptoms. SARS-CoV-2 virus can cause almost any neurological disease, and relapse of KLS is another evidence of neurotropicity of the virus.
10.3390/neurolint13030033
What respiratory physicians should know about narcolepsy and other hypersomnias.
Breathe (Sheffield, England)
Narcolepsy and related central disorders of hypersomnolence may present to the sleep clinic with excessive daytime sleepiness. A strong clinical suspicion and awareness of the diagnostic clues, such as cataplexy, are essential to avoid unnecessary diagnostic delay. This review provides an overview of the epidemiology, pathophysiology, clinical features, diagnostic criteria and management of narcolepsy and related disorders, including idiopathic hypersomnia, Kleine-Levin syndrome (recurrent episodic hypersomnia) and secondary central disorders of hypersomnolence.
10.1183/20734735.0157-2022
Kleine-Levin syndrome with comorbid iron deficiency anemia.
Jain Rajendra Singh,Kumar Sunil,Srivastava Trilochan,Sannegowda Raghavendra Bakki
Oxford medical case reports
Kleine-Levin syndrome (KLS) is a rare chronic sleep disorder of unknown etiopathology, which typically occurs in adolescent males. Although the severity of symptoms and disease course varies between the KLS patients, it usually resolves spontaneously, but sometime comorbid conditions may worsen the symptoms. Herein, we report a case of KLS who presented with severe episodic hypersomnia. During episodes, the patient used to sleep as long as 20 h in a day, affecting his daily living activities. All the relevant investigations including electroencephalography, magnetic resonance imaging of brain and cerebrospinal fluid analysis were normal except for severe iron deficiency anemia (IDA). In our patient, the severity of symptoms worsened due to coexistent IDA. The treatment of IDA along with modafinil decreased the severity of symptoms and shortened the hospital stay during episodes. This might be the first case report of KLS with comorbid IDA.
10.1093/omcr/omv019
IV steroids during long episodes of Kleine-Levin syndrome.
Léotard Antoine,Groos Elisabeth,Chaumereuil Charlotte,Peter-Derex Laure,Rossetti Andrea O,Leu-Semenescu Smaranda,Arnulf Isabelle
Neurology
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.
10.1212/WNL.0000000000005349
A Polysomnography Study of Kleine-Levin Syndrome in a Single Center.
Luo Yan-Wen,Yu Huan,Yuan Lu-Hua,Zhu Guo-Xing
Chinese medical journal
BACKGROUND:Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of hypersomnia. Polysomnographic (PSG) researches of KLS have been reported only in few publications in the past decades. This study aimed to investigate the characteristics of PSG of KLS. METHODS:This study, which was conducted from March 2010 to July 2014, included seven patients diagnosed with KLS in the Sleep and Wake Disorder Center of Huashan Hospital, Fudan University (Shanghai, China). PSG and multiple sleep latency tests (MSLT) were performed during their episodes and the results were evaluated. RESULTS:Five of the seven patients were males. The mean age at KLS onset was 15.6 ± 3.6 years. The number of episodes ranged from 2 to 7. The duration of episodes lasted from 4 to 11 days. The sleep architecture and proportion were normal in most of the patients. The average value of mean sleep latency was 6.9 ± 4.1 min. No sleep-onset rapid eye movement (SOREM) was detected in three of the patients, whereas one patient experienced one period of SOREM, and such episodes occurred twice in other two patients. CONCLUSIONS:We found that sleep architecture and proportion were normal in most KLS patients. However, the results of PSG and MSLT had no specificity for KLS patients.
10.4103/0366-6999.184476
Kleine-Levin syndrome: an autoimmune hypothesis based on clinical and genetic analyses.
Dauvilliers Y,Mayer G,Lecendreux M,Neidhart E,Peraita-Adrados R,Sonka K,Billiard M,Tafti M
Neurology
BACKGROUND:Kleine-Levin syndrome (KLS) is a rare disorder of unknown etiology. Pathophysiologic hypotheses include a hypothalamic dysfunction and abnormalities in the central serotonin and dopamine metabolism. Several clinical symptoms also suggest an underlying autoimmune process. OBJECTIVE:To systematically investigate patients with KLS with reference to the available hypotheses. METHODS:The authors collected clinical, polysomnographic, CSF, CT, and MRI records and analyzed gene polymorphisms of HLA-DQB1, tryptophan hydroxylase (TpH), and catechol-O-methyltransferase (COMT) in 30 unrelated patients with KLS and their families. The genotype data were contrasted with data from a normal control population. RESULTS:Only human leukocyte antigen (HLA)-DQB1*0201 allele frequency was significantly increased in patients with KLS. Three patients with KLS but none of the control subjects were DQB1*0201 homozygous. Two affected subjects from the same family were DQB1*0201 homozygous. In 17 DQB1*0201 heterozygous parents, 11 (64.7%) had transmitted this allele, suggesting a preferential transmission. CONCLUSION:These findings, together with the young age at onset, the recurrence of symptoms, and the frequent infectious precipitating factors, suggest an autoimmune etiology for Kleine-Levin syndrome.
10.1212/01.wnl.0000036605.89977.d0
[Clinical presentation of Kleine-Levin syndrome].
Hu Y,Wang J Y,Dong X S,Li J,Yan H,Wang P P,Zhao L,Zhang X Z,Han F
Zhonghua yi xue za zhi
To analyze the clinical features of Kleine-Levin syndrome (KLS) patients. Clinical data of 44 patients with KLS of the Sleep Center of Peking University People's Hospital from January 2002 to July 2013 were systematically reviewed. The predisposing factors and clinical presentations were summarized, and compared with the data from a Western KLS study with a large subjects number. Nocturnal polysomnography (PSG) and multiple sleep latency test (MSLT) were conducted during relapse and remission period, respectively. HLA-DQB1*0602 gene were screened and analyzed. Among the 44 patients, 28(63.6%) were men and 16(36.4%) were women, with a mean age of (18.3±8.9) years old. Most patients developed the symptoms during adolescence. Infection or fever was the most common trigger for episode. The main clinical presentations were 44(100.0%) hypersomnia, 31(70.5%) forgetfulness, 26(59.1%) decreased appetites, 24(54.5%) juvenile behavior, 18(40.9%) depression, etc. Compared with the Western study, it showed that our patients had decreased instead of increased appetite. The PSG testing did not have remarkable findings. MSLT showed mean sleep latency was significantly shorter during relapse than during remission [(10.4±5.4) vs (15.3±3.4) min, =0.009]. HLA-DQB1*0602 was positive in 12 of 40(30.0%) patients, which was similar to the data in the Chinese population. KLS has various clinical characteristics. The presentation of appetite may be different between Chinese and western KLS patients.
10.3760/cma.j.issn.0376-2491.2017.16.011
Treatment of Kleine-Levin Syndrome With Intranasal Photobiomodulation and Methylene Blue.
Hamper Michael,Cassano Paolo,Lombard Jay
Cureus
Kleine-Levin syndrome (KLS) is a rare neuropsychiatric disorder, characterized by recurrent episodes of idiopathic hypersomnia, and cognitive and behavioral abnormalities, such as memory loss and child-like language. There is no definitive etiology for KLS; however, there are hypotheses of genetic predisposition, autoimmune mechanisms, and abnormal thalamic and hypothalamic functioning. Similarly, there is no definitive treatment for KLS as one method may be beneficial for one patient and not for another. We present a case of KLS in a patient who has no clinical improvement in symptoms with a variety of treatments. The parents of the patient agreed to attempt a trial of intranasal photobiomodulation (i-PBM) with red light, in combination with methylene blue (MB). The patient showed remission of the KLS episode following treatment with no further KLS episodes reported after treatment.
10.7759/cureus.18596
Genetics and epigenetics of rare hypersomnia.
Trends in genetics : TIG
Herein we focus on connections between genetics and some central disorders of hypersomnolence - narcolepsy types 1 and 2 (NT1, NT2), idiopathic hypersomnia (IH), and Kleine-Levin syndrome (KLS) - for a better understanding of their etiopathogenetic mechanisms and a better diagnostic and therapeutic definition. Gene pleiotropism influences neurological and sleep disorders such as hypersomnia; therefore, genetics allows us to uncover common pathways to different pathologies, with potential new therapeutic perspectives. An important body of evidence has accumulated on NT1 and IH, allowing a better understanding of etiopathogenesis, disease biomarkers, and possible new therapeutic approaches. Further studies are needed in the field of epigenetics, which has a potential role in the modulation of biological specific hypersomnia pathways.
10.1016/j.tig.2023.02.003
Kleine-Levin syndrome elicited by encephalopathy with reversible splenial lesion.
Takayanagi Masaru,Okabe Shinichi,Yamamoto Katsuya,Komatsu Juri,Suzuki Rikio,Kitamura Taro,Ohura Toshihiro
Pediatrics international : official journal of the Japan Pediatric Society
Kleine-Levin syndrome is a rare sleep disorder of unknown etiology characterized by repetitive episodes of hypersomnia between asymptomatic periods. We report the case of a 13-year-old girl who presented with drowsiness triggered by influenza A as the first episode. Magnetic resonance imaging (MRI) on day 6 showed transient reduction of diffusion in the corpus callosum splenium. The patient was diagnosed with encephalopathy with a reversible splenial lesion. The symptoms resolved after 10 days, but additional episodes of hypersomnia lasting 5-10 days occurred 1, 5, 6, 11, 13, and 25 months after the first episode. MRI during hypersomnia indicated no lesions, and sleep duration and cognition were normal between episodes. The patient was diagnosed with Kleine-Levin syndrome. Electroencephalographic and clinical findings during the first episode were similar to those during the other episodes. Encephalopathy with a splenial lesion and Kleine-Levin syndrome may have similar pathological mechanisms causing a disturbance in consciousness.
10.1111/ped.13326