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LETTER TO EDITOR
Year : 2009  |  Volume : 4  |  Issue : 1  |  Page : 47-48
 

An adolescent girl with periodic hypersomnolence and megaphagia


NEIGRIHMS, North Eastern Indira Gandhi Regional Institute of Medical Sciences, Shillong, India

Correspondence Address:
S R Sharma
NEIGRIHMS, North Eastern Indira Gandhi Regional Institute of Medical Sciences, Shillong
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.49110

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How to cite this article:
Sharma S R, Sharma N. An adolescent girl with periodic hypersomnolence and megaphagia. J Pediatr Neurosci 2009;4:47-8

How to cite this URL:
Sharma S R, Sharma N. An adolescent girl with periodic hypersomnolence and megaphagia. J Pediatr Neurosci [serial online] 2009 [cited 2019 Aug 19];4:47-8. Available from: http://www.pediatricneurosciences.com/text.asp?2009/4/1/47/49110


Sir,

Klein-Levin syndrome, usually found in adolescent males, is associated with episodic hypersomnolence, compulsive overeating, lack of sexual inhibition, and personality change, and most often, it represents a benign and self-limited entity and does not warrant extensive investigation or treatment. Female affection of the disease is very rare.

A 13 year-old girl was brought by her parents with a complaint of periodic hypersomnolence. Her parents were concerned that her periodic hypersomnolence would affect her examinations. On further enquiry, she was found to have a history of sleeping for 18 to 20 hours a day, which would happen for continuously 6-7 days, seven to eight times a year for the past two years. Apparently, she had no warning symptoms and could not predict when she would have such episodes. In addition to sleeping for 18 to 20 hours a day, she was also reported to eat voraciously while awake. If woken up from sleep, she was rather irritable and went back to sleep quickly. Barring these episodes, she was a normal child with normal intelligence and behavior. She ranked in the top ten percentile in her class and had no other significant medical history. In particular, there was no history of trauma, seizure-like activity, snoring, or hypersomnolence at other times. Her age at menarche was 12 years. There was no significant family or psychosocial history.

On examination, her weight and height were found to be normal for her age. Results for investigations done prior to referral to the Sleep Center, including liver functions, renal functions, thyroid functions, blood sugar, growth hormone, prolactin levels, luteinizing hormone, follicular stimulation hormone, GnRH, and estradiol were all normal. The results of her MRI, EEG, and LP exam were normal. A 16 channel overnight polysomnography was done and found to be normal. In particular, the sleep architecture was normal and there was no evidence of sleep-disordered breathing or periodic limb movement disorder. A Multiple Sleep Latency test done the following day did not suggest hypersomnolence and there were no sleep-onset REM periods. Of note, the study was done during the days that were reported to be the patient's normal days and not during one of her 'periodic' abnormality, as the frequency of these was unpredictable. A diagnosis of Klein-Levin syndrome (KLS) was made. It was decided to start her on lithium (400-900 mg/day bid or at night) and to monitor the frequency of her symptoms.

Klein-Levin syndrome is a rare disorder characterized by the need for an excessive amount of sleep (hypersomnolence), ( i.e ., up to 20 hours a day); excessive food intake (compulsive megaphagia); and an abnormally uninhibited sexual drive occurring almost exclusively in adolescent males, though there have been a few reports of its occurrence in females as well. [1] The mean age of onset is 15.8 2.5 years and usually patients experience three to four episodes per year with a mean duration of 11.5 6.6 days for a single hyper somnolence attack. [2] It has been noted that the disease usually lasts 8-10 years and is self limiting before adulthood.

Although the cause is usually not known, it is believed that hereditary factors may cause some individuals to have a genetic predisposition to developing this disorder. There have been reports of two siblings who shared uncharacteristically prolonged episodes of hypersomnolence and the HLA-DR2 haplotype [3] Secondary Klein-Levin syndrome has been described after episodes of encephalitis [4] and trauma. [5],[6] Differential diagnoses included seizure disorder and depression.

KLS is usually associated with compulsive overeating, lack of sexual inhibition, and personality change. Sexual responses though not always present include in appropriate sexual advances and overt masturbation, especially in males. Compulsive overeating with rapid weight gain may occur. Personality changes may include irritability, depersonalization, depression, confusion, occasional hallucinations, and impulsive behavior; the symptoms of KLS are typical. On recovery, total or partial loss of memory (amnesia) is usual, although disgust at overeating is common. Between episodes, physical and mental health is usually normal.

It is not uncommon to note that these patients are seen by a variety of specialists including endocrinologists, psychiatrists, and neurologists before being evaluated by a sleep specialist. This meant a delay in diagnosing Klein-Levin syndrome of 3.8 4.2 years. [2]

Results of all imaging tests, except of SPECT, are usually normal. The subtracted SPECT studies may show hypoperfusion in the left hypothalamus, bilateral thalami, basal ganglia, bilateral medial and dorsolateral frontal regions, and the left temporal lobe during the symptomatic period. [7] CSF hypocretin-1 measurements performed during the periods of hypersomnia have also been found to be abnormal . [8]

Currently, there is no formal treatment for KSL due to the lack of knowledge regarding its underlying cause. Only lithium [9] has had a higher reported response rate (41%) for stopping relapses when compared to medical abstention (19%). The goal of treatment is to counsel the patient and family and also, to keep these episodes to a low level throughout the year.

Most of these patients are either labeled as being lazy or classified as narcolepsy or idiopathic hypersomnia. But the absence of associated clinical features such as cataplexy, and characteristic polysomnographic features such as sleep-onset REM episodes and the presence of megaphagia facilitate the positive diagnosis.

It is important that the diagnosis is suspected early, especially in adolescent males who present with recurrent episodes of somnolence, increased appetite, and abnormal behavior, as it most often represents a benign and self-limited entity and does not warrant extensive investigation or treatment. It is also important to distinguish this syndrome from more serious organic and psychiatric disorders with more serious prognosis. KLS remains particularly rare in girls and is challenging to diagnose as most girls have no prior physical, neurological, or psychiatric history.

 
   References Top

1.Kesler A, Gadath N, Veinstein G, Peled R, Lavie P. Kleine Levin syndrome (KLS) in Young females. Sleep 2000;23:563-7.  Back to cited text no. 1    
2.Gadoth N, Kesler A, Vainstain G, Peled R, Lavie P. Clinical and polosomnographic characteristics of 34 patients with Kleine Levin Syndrome. J Sleep Res 2001;10:337-41.  Back to cited text no. 2    
3.Katz JD, Ropper AH. Familial Kleine-Levin syndrome: Two Siblings with unusually long hypersomnic spells. Arch Neurol 2002;59:1959-61.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Sethi S, Bhargava SC. Kleine-Levin syndrome and encephalitis. Indian J Pediatr 2002;69:999-1000.  Back to cited text no. 4  [PUBMED]  
5.Cheung G. Posttraumatic Kleine-Levin syndrome. Gen Hosp Psychiatry 2006;28:443-5.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Pelin Z, Ozturk L, Bozluolcay M. Posttraumatic Kline-Levin syndrome: A case report. Eur Psychiatry 2004;19:521-2.  Back to cited text no. 6    
7.Huang YS, Guilleminault C, Kao PF, Liu FY . SPECT findings in Klein-Levin syndrome. Sleep 2005;28:955-60.  Back to cited text no. 7    
8.Podestα C, Ferreras M, Mozzi M, Bassetti C, Dauvilliers Y, Billiard M. Kleine-Levin syndrome in a 14-years-old girl: CSF hypocretin-1 measurements. Sleep Med 2006;7:649-51.  Back to cited text no. 8    
9.Muratori F, Bertini N, Masi G. Efficacy of Lithium treatment in Kleine-Levin syndrome. Eur Psychiatry 2002;17:232-3.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]




 

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