|Year : 2019 | Volume
| Issue : 1 | Page : 58-59
Kleine-levin syndrome: A rare case and review
Falguni B Patel, Parlin M Dadhaniya, Manasvi V Jariwala, Bhaveshkumar M Lakdawala
Department of Psychiatry, AMC MET Medical College and Sheth L. G. General Hospital, Ahmedabad, Gujarat, India
|Date of Web Publication||24-May-2019|
Dr. Bhaveshkumar M Lakdawala
Department of Psychiatry, AMC MET Medical College and Sheth L. G. General Hospital, Maninagar, Ahmedabad - 380 008, Gujarat
Source of Support: None, Conflict of Interest: None
Kleine–Levin syndrome (KLS), also known as sleeping beauty syndrome, is a rare disorder with unknown etiology. The syndrome is characterized by hypersomnolence and may be associated with hypersexuality, hyperphagia and cognitive or mood changes. Fever, head trauma and stress may be the precipitating factors. The case here is a 15-year-old male patient who presented with excessive sleepiness, hypersexual behavior, excessive food intake and mood changes. These symptoms started after becoming afebrile from high-grade fever. Multiple investigations done were within normal limits. He did not respond to valproate, olanzapine and lorazepam. Spontaneous remission and reemergence of symptoms after 2 weeks were noted. By clinical history, normal investigations and exclusion of other mental and medical disorders, he was diagnosed to have KLS. Reviewing literature, lithium therapy was found to be beneficial in KLS. Hence, lithium was given in this patient to which he responded well.
Keywords: Hypersomnolence, Kleine–Levin syndrome, lithium, mood disturbance
|How to cite this article:|
Patel FB, Dadhaniya PM, Jariwala MV, Lakdawala BM. Kleine-levin syndrome: A rare case and review. Ann Indian Psychiatry 2019;3:58-9
|How to cite this URL:|
Patel FB, Dadhaniya PM, Jariwala MV, Lakdawala BM. Kleine-levin syndrome: A rare case and review. Ann Indian Psychiatry [serial online] 2019 [cited 2019 Aug 20];3:58-9. Available from: http://www.anip.co.in/text.asp?2019/3/1/58/259093
| Introduction|| |
Kleine–Levin syndrome (KLS) is a rare disorder with an incidence of one in 1 million characterized by recurrent excessive episodic hypersomnolence and associated with sexual disinhibition, hyperphagia and mood changes. It was first reported by Kleine and Levin. KLS has unpredictable course, remissions and recurrences. A review article found a median duration of 8 years. Due to rarity of the disorder, symptoms of KLS may be misdiagnosed as a major psychiatric disorder. Not much literature is available on KLS and its treatment. The case presented here would be helpful for the evaluation and diagnosis of such cases in future.
| Case Report|| |
A 15-year-old male patient, studied up to 8th standard, working as a fruit vendor, coming from urban background with uneventful birth and developmental history, no past and family history of psychiatric illness presented with complaints of marked irritability, abusiveness, hypersexual behavior and increased sleep with 15 days duration. Hence, he was admitted in the psychiatry ward for detailed evaluation and confirmation of diagnoses. The patient had a history of fever before 15 days, for which he was admitted to a private hospital; however, no specific cause for fever was found. He was treated by a physician with intravenous fluids and antipyretics and was discharged after 3 days as he was afebrile. Then, parents observed abnormal behavior in the form of decreased interaction with the family members. He was sleeping for most of the time of the day, i.e., around 16–18 h a day, as compared to his routine of around 8–9 h at night time only. He was getting irritable without provocation and used abusive language when he was awake. Relatives also observed his sexual behavior such as rubbing and touching his private parts in front of anyone after 5–6 days of onset of illness. He was taking excessive food and would eat everything offered to him and also gained 5 kg weight (47–52 kg) in 15 days as reported by parents. Such behavior was never observed previously by relatives. Occasionally, he was giving irrelevant answers to questions asked and was unable to remember such behavior. The patient complained of headache, backache, and pain in the fingers and toes. He was subjected to routine laboratory investigations such as blood counts, renal and liver function tests, thyroid function test and computed tomography scan of the brain. These investigations were within normal limits. Considering provisional diagnosis to be bipolar mood disorder, sodium valproate up to 1000 mg and olanzapine up to 10 mg were started. However, no improvement was observed with the treatment even after 2 weeks. Then, sudden remission of all symptoms occurred in 1–2 days, and the patient was discharged. The patient was asymptomatic for the next 2 weeks. Then, he again presented with similar complaints and was admitted again. Neurologist's opinion was taken, and as per his advice, magnetic resonance imaging of brain (plain), electroencephalography, cerebrospinal fluid examination and sleep study were done and found to be normal. Thus, after ruling out other medical causes and mental disorders, and on the basis of clinical history, we diagnosed the patient to have KLS. Lithium was started from 600 mg per day with which only mild improvement in irritability and abusiveness was observed and so dose was increased after a week up to 900 mg per day which was effective as the patient improved significantly in all presenting symptoms. The patient is better at present for the past 24 months.
| Discussion|| |
KLS is a rare neuropsychiatric disorder with male predominance, onset during adolescence and spontaneous remission of symptoms. The frequency of episodes varies from 1 to 12 in a year. The International Classification of Sleep Disorder-3 has defined criteria for KLS. Psychotic symptoms and mood changes can be a part of this syndrome. Hence, it is easily misdiagnosed as a major mental disorder. Therefore in a case with episodic hypersomnolence, KLS should be suspected after ruling out other differentials such as bipolar mood disorder, atypical depression, seasonal affective disorders, substance use, narcolepsy, focal brain lesions, Kluver–Bucy syndrome, viral encephalitis and temporal lobe epilepsy. In this case, the patient was subjected to various investigations which ruled out other causes. Hypothalamic dysfunction, abnormalities in serotonin and dopamine metabolism and autoimmune process may be pathophysiologic hypothesis. Precipitating factors such as fever, head trauma, encephalitis and stress can be present. The usual onset with febrile illness and the inflammatory lesions detected in the hypothalamus and thalamus by Carpenter et al. suggest a possible viral etiology. In the case presented here, the first episode was preceded by febrile illness. The diagnosis was done on the basis of detailed clinical evaluation. There is no specific, definitive treatment to cure or control KLS. Lithium is useful in KLS as can be ascertained from various case reports., In this case also, the patient benefitted from lithium therapy, and so, it is continued. Patient education, family education and counseling were done about the nature and course of the disease, how to recognize the episode of KLS, and also the need for family support to the patient.
| Conclusion|| |
In conclusion, it is evident that KLS causes significant impairment in the different areas of functioning of the patient and also causes distress to the family members. As KLS is misdiagnosed and managed as other sleep disorder, psychiatric disorder or neurological disorder, it is essential to suspect and evaluate for KLS in such cases having atypical presentation for early diagnosis and rational treatment, which can alleviate the suffering of both the patient and their relatives. However, it is also clear that there are no specific biomarkers, laboratory findings or neuroimaging studies for the diagnosis of KLS, making it a diagnostic challenge.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Tiwari A, Agarwal A, Bharti S, Kohli A. Kleine-Levin syndrome: A case report. Int J Adv Med 2017;2:54-5.
Arnulf I, Zeitzer JM, File J, Farber N, Mignot E. Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain 2005;128:2763-76.
Murthy VS, Kelkar AD, Sonavane SS. Psychosis in a case of Kleine-Levin syndrome: A diagnostic challenge. Indian J Psychol Med 2014;36:192-4.
] [Full text]
Critchley M. Periodic hypersomnia and megaphagia in adolescent males. Brain 1962;85:627-56.
American Academy of Sleep Medicine. International Classification of Sleep Disorders: Diagnostic and Coding Manual. 3rd
ed. Westchester, Illinois: American Academy of Sleep Medicine; 2013.
Lavault S, Golmard JL, Groos E, Brion A, Dauvilliers Y, Lecendreux M, et al.
Kleine-Levin syndrome in 120 patients: Differential diagnosis and long episodes. Ann Neurol 2015;77:529-40.
Carpenter S, Yassa R, Ochs R. A pathologic basis for Kleine-Levin syndrome. Arch Neurol 1982;39:25-8.
Kellett J. Lithium prophylaxis of periodic hypersomnia. Br J Psychiatry 1977;130:312-3.
Roth B, Smolík P, Soucek K. Kleine-Levin syndrome–Lithium prophylaxis. Cesk Psychiatr 1980;76:156-62.