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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 4 | Page : 873-878
Kleine–Levin syndrome: A neurological rarity
Department of Neurophysiology and Sleep Medicine, Sir Ganga Ram Hospital, New Delhi, India
|Date of Web Publication||5-Jul-2017|
Consultant Neurologist, Department of Sleep Medicine and Neurophysiology, Sir Ganga Ram Hospital, Rajender Nagar, New Delhi - 110 060
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Panda S. Kleine–Levin syndrome: A neurological rarity. Neurol India 2017;65:873-8
This is a report of a 17-year old school boy presenting with episodes of excessive sleepiness since the age of 14 years. Though initially rare, its frequency had increased one year prior to presentation (a total of 7 episodes), lasting for 10–12 days, during which he would get up only for food and voiding. Prodromal cephalic feeling preceded hypersomnolence by 1–2 days. Despite his extreme craving for food, he could abstain from eating too much to avoid undue weight gain. He was irritable, wanted to lie down in quiet surroundings, and could not tolerate sound or light. He had difficulty in finding objects and would be clumsy and confused even 2–3 days prior to the episode. There was no disturbance in language, speech, nor did he have a hypersexual behavior. He was noticed to have frequent movements of legs during sleep, but had no history of aura, snoring, sleep paralysis, vivid dreams, sleep fragmentation, or sudden drop attacks. No preceding history of acute infection, fever, head trauma, or drug intake was noted. There was no history of stress, psychological distress, or obsessive behavior. The boy had normal mental abilities with an excellent academic record and was actively participating in sports and extracurricular activities. He had tried modafinil and ayurvedic treatment previously without any benefit. He had a single febrile seizure at 2.5 years of age and a probable complex partial seizure following a hot water bath at 13 years of age; however, he was not on long-term antiepileptic drugs.
General physical and systemic examination revealed no abnormality except for drowsiness at the initial visit. Electroencephalogram (EEG) done 6 days after the onset of episode showed intermittent left temporal slow and sharp waves without electrographic seizures or nonconvulsive status epilepticus [Figure 1]a, and magnetic resonance imaging (MRI) of the brain revealed no abnormality [Figure 1]b. The patient refused to undergo polysomnography. A clinical diagnosis of Kleine–Levin syndrome (KLS) was made, and he was started on carbamazepine at 400 mg per day, on which he remained event free for a period of 2 years.
|Figure 1: (a) EEG showing sharp waves in the left temporal region; (b) Normal T1 weighted image of MRI brain|
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KLS is a rare and enigmatic disorder, demonstrating the involvement of physiologically interdependent functions of sleep, appetite, and food intake with symptoms of psychological disorders. Its rarity (prevalence of 1–2 per million), symptomatic expression, unclear etiopathogenesis, and lack of a biomarker, makes it difficult to diagnose this entity. After the initial descriptions of KLS at the turn of the 20 th century, Critchley first wrote about this disorder in 1942, which was having the characteristic hallmarks of “morbid hunger and periodic somnolence.” He assigned it the nomenclature of the “Kleine–Levin syndrome” in 1962. The diagnostic criteria for KLS have been recently modified by the International Classification of Sleep Disorders-3 in 2013. The prevalence of the KLS has been described worldwide with a predominance in Europe and Asia, with a Jewish predisposition and a male–female ratio of 3:1.
There are 48 cases of KLS reported from India [Table 1]. Thirty-seven (77%) patients were male, with a male–female ratio of 3.2:1. The age of onset of KLS ranged from 10 to 25 years (the majority being in the second decade), as was evident in our patient. Unlike the index case, secondary KLS is more common and occurs following triggering stimuli, such as infection or fever, alcohol or marijuana intake, head trauma, sleep deprivation, stress, psychological distress, physical exertion, menstruation, or lactation. In this Indian review, the most frequent precipitating factor was a brief episode of fever or a probable viral infection, with the symptoms of the KLS occurring shortly after (between 2 and 6 days) the onset of fever.,, Other precipitating factors that have been reported have included stressful events such as marital disharmony, fight with husband or in-laws, approaching exams, or the postpartum period. KLS lasted for 2 weeks to 12 years with 1–25 episodes, each lasting for 1–14 days [Table 1].,,,,,,,,,,,, While hypersomnia is mandatory for its diagnosis, an incomplete presentation is usually more common.,,,,,,, Changes in eating behavior, especially megaphagia, were noted in all except three patients. One patient was hospitalized for breathlessness, resulting from a distended abdomen, after having a large meal; 2 patients would “grab any food in sight;” a vegetarian patient ate nonvegetarian food during the episodes; and, a patient even chewed pieces of paper. On the contrary, 12 patients were reported to have anorexia., Our patient had extreme craving for food with ability to control this urge, an awareness not seen in any of the previously reported cases.
|Table 1: Clinical characteristics of patients with Kleine-Levine syndrome in reported Indian literature|
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While irritability was present in almost all the patients, especially when sleep, sexual, or food drive was prohibited, hypersexual behavior or social disinhibition was not forthcoming in our patient and in the patients included in some reports from India.,,,,,,, On the contrary, severe aggressive behavior was noted in an otherwise shy boy, while a man was caught by police for throwing stones, 2 patients had visual and auditory hallucinations, 2 had excessive crying, and 1 had comorbid obsessive-compulsive disorder., Some behavioral features not previously reported in KLS but seen in the index case include a prodromal sensation, confusion during the episodes, intolerance to light and sound with difficulty in finding objects, and coexistent epilepsy (except in 3 patients ) [Table 1].
The treatment includes central nervous system (CNS) stimulants such as amphetamines, modafinil, methylphenidate, and neuroleptics; mood stabilizers such as lithium; antiepileptics such as carbamazepine and valproate; and other agents – melatonin, phototherapy, antivirals, antidepressants, benzodiazepines, and electroconvulsive therapy. The prognosis of KLS is considered to be good with spontaneous remission in most of the cases in later years. However, a high index of suspicion is warranted as KLS poses a diagnostic as well as a therapeutic challenge.
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