Department of Neurology, Nagano Municipal Hospital, Japan. minori_kodaira@hospital.nagano.nagano.jp
Abstract
Six days after the onset of influenza B symptoms, a 14-year-old Japanese boy presented with encephalopathy-like symptoms, somnolence, irritability, and childishness, which we first considered was an atypical type of influenza-associated encephalopathy because the infection symptoms disappeared by day 4. His encephalopathy-like symptoms gradually improved, although he had repetitive hypersomnia attacks. Owing to the patient’s clinical presentation and normal interleukin-6 levels in the cerebrospinal fluid during the first period of hypersomnia, we diagnosed him with Kleine-Levin syndrome (KLS) triggered by influenza B. The preceding influenza infection was not only a diagnostic clue of KLS but also a diagnostic confounding factor.
Department of Neurology, Hull Royal Infirmary, Hull, UK.
Abstract
Episodic hypersomnia, compulsive excessive eating and erotic behaviour, with schizophrenic-like mental symptoms are the hallmarks of the rare Kleine-Levin syndrome. This paper traces the origins of its description back to the 18th century and the changing concepts of its complex organic-psychogenic aetiology. The eventual, spontaneous disappearance of the syndrome is unexplained.
A case of Kleine-Levin syndrome in a fourteen year old junior high school female student is described. The episodic disorder was characterised by excessive sleep, voracious appetite, irritability, confusion and electro-encephalographic changes. Remission was spontaneous. Prospects for organic aetiology, differential diagnosis, and the importance of early detection are discussed.
Toby Foster meets the teenager who spends much of his life asleep. Mitchell has the rare condition of Kleine Levin Syndrome (KLS) – a rare sleeping and behavior disorder which is episodic and robs teenagers of their time, my own son is also has KLS – he is a year younger than Mitchell and slept for over half of the last year.
More information on KLS can be found on my web site – http://www.renoufdesign.co.uk/KLS along with a leaflet which I have designed, approved by my consultant to help promote understanding of this horrible condition.
Alanna Wong on Anderson Cooper Show. Extreme Sleep Disorders. Alanna has Kleine-Levin Syndrome (KLS). Please visit http://www.klslife.com for more information.
What is KLS?
What is KLS?
Kleine-Levin Syndrome is a rare neurological disorder which affects roughly 1000 known patients worldwide. It is a life-altering illness which is widely misunderstood and frequently misdiagnosed as a psychological illness. The illness often strikes in adolescence and many sufferers exhibit symptoms for years before the illness appears to taper off. KLS is a devastating illness which robs the patient of time, experiences, and relationships.
KLS is episodic, meaning that the patient alternates between sick and healthy states. Episodes vary in duration and can last days, weeks, months, or even years. When in an episode, a KLS sufferer functions in a dream-like or altered state and can exhibit periods of excessive sleep and altered personality traits. In between episodes the patient seems normal with no outward signs of behavioral dysfunction. When healthy, a patient may not have much memory of the time spent ill.
During a KLS episode, blood flow is reduced to the thalamus region of the brain, particularly the hypothalamus which regulates the basic human drives of fight, flight, food, and sex. The decreased blood flow is either the cause or effect of KLS resulting in physical and behavioral changes such as excessive sleep and hunger, altered personality, and a dream-like state. When supporting a KLS sufferer it is important to remember that behavioral changes are not a choice but are in fact caused by a change in brain function.
KLS is a devastating illness. Family and friends can provide crucial support and comfort by acknowledging the illness and letting the sufferer know that they are loved. Understanding, loyalty, and friendship can help a patient through difficult times.
Eponym:
Willi Kleine and Max Levin are doctors that in the early 1900s described cases of recurrent hypersomnolence and hyperphagia with altered behavior- hence Kleine Levin Syndrome.
CAUSE
The cause of Kleine-Levin Syndrome is still unknown. Some theories suggest that KLS could be caused by a viral or bacterial component while others explore the possibility of a genetic susceptibility to KLS. There are also those who believe that KLS may be affected by the immune system. It is possible that the illness is caused by a combination of factors. The following is a link to the KLS foundation Status of KLS Research at Stanford University:
Division of Neurology, Department of Medicine, Phramongkutklao Hospital and Medical College, Bangkok, Thailand. pasiripmk@gmail.com
Abstract
Kleine-Levin syndrome (KLS) is a rare disorder characterized by periodic hypersomnia, cognitive and behavioral disturbances. Other unique symptoms in KLS are megaphagia, hypersexuality and some psychiatric disturbances such as compulsion and depression. Definite diagnosis requires the elimination of other potential etiologies. We reported a typical case of KLS in a young Thai man who suffered from seven episodes of periodic hypersomnia within 1.5 years and eventually he was diagnosed with Kleine-Levin syndrome after excluding known possible neurological conditions and sleep disorders.
Toby Foster meets the teenager who spends much of his life asleep. Mitchell has the rare condition of Kleine Levin Syndrome (KLS) – a rare sleeping and behavior disorder which is episodic and robs teenagers of their time, my own son is also has KLS – he is a year younger than Mitchell and slept for over half of the last year.
More information on KLS can be found on my web site – http://www.renoufdesign.co.uk/KLS along with a leaflet which I have designed, approved by my consultant to help promote understanding of this horrible condition.
My son began to sleep more in August 2011. He was 13 years old and we were on holiday, at first we thought he banged his head and had concussion, but he was not right for a long time afterwards, and the Autumn was a long difficult battle trying to find out what was wrong. He was in episode for nearly 4 months. In December he was diagnosed with Kleine Levin Syndrome (KLS) he was still in this episode – he came out of it just before Christmas 2012, he had no memory of anything since the summer.
Those first four months saw us fighting an unknown battle, our son was sleeping excessively, always exhausted and operating in a dream… he was unable to do some things but had an incredible memory – it was like he was overloading his mind with data. Some people were suggesting he was just a lazy teenager but it was so much more. We saw our GP, a neurologist, psychiatrist, visited a private clinic in Harley Street, had various tests and were being passed from pillar to post with our “interesting case” that no one recognised and we were getting more and more desperate as parents. He was finally diagnosed by letter – we had wrote a detailed letter about his condition – all the signs and symptoms the doctors were just not getting, and this letter landed on a desk of a fantastic paediatrician who read it, and recognised the symptoms from a conference she had attended in the year – what we described was not in the text book, but was KLS – a week later we saw her, had a provisional diagnoses and shortly after that our first episode ended… our son was surprised to realise it was Christmas, we celebrated together and then he went into his second episode before New Year – practically confirming the episodic nature of the condition.
I have started this page a year on – it is August 2012. My son has an extreme case of KLS – he has had 15 episodes in his first year, they have varied in length from half a day to 4 months. He has missed 180.5 days, 12.5 days have been “foggy” and he has had 171 good days where life has gone on. He is in year 9 at school – is on a revised timetable and despite only spending 78 days in school (most of them half days) he has actually caught up with his main subjects.
Division of Child Neurology and Psychiatry, University of Pisa, Italy. masi@inpe.unipi.it
Abstract
The Kleine-Levin syndrome (KLS) is characterized by periodic, sudden-onset episodes of hypersomnia, compulsive hyperphagia, and behavioral-emotional disorders (typically indiscriminate hypersexuality, irritability, impulsive behaviors), lasting from a few days to a few weeks, with almost complete remission in the intercritical periods. Depression, confusion, and thought disorders are frequently associated with the critical symptomatology, and they may suggest other psychiatric diagnoses (schizophrenia, mood disorder, conversion disorder) or a substance abuse. A diencephalic-hypothalamic dysfunction is suspected, even if this composite symptomatology cannot easily be linked to a simple mechanism. The aim of this article is to illustrate problems in differential diagnosis, using a case approach. History, course, and therapeutic intervention in a 21-year-old patient with KLS, associated with a clear psychiatric symptomatology and a critical affective pattern, is reported. Psychiatric correlates of KLS are discussed, including the relationship with affective disorders and the possible emotional impact of the attacks. Implications regarding a combined psychological and pharmacological treatment are also discussed.
Jeune équipe Ethique, Professionnalisation, Santé (JE 2535), université de Bretagne occidentale, UFR médecine et sciences de la santé, Service hospitalo-universitaire de psychiatrie de l’enfant et de l’adolescent, hôpital de Bohars, CHU de Brest, France. richard@clinique-valjosselin.fr
Abstract
The purpose of this article is to report an original clinical case whose symptoms suggest a very peculiar pathology, because of its rarity, symptomatic expression and unclear etiopathogenesis: the Kleine-Levin Syndrome (KLS). During the regression of tonsillitis concomitant with an emotional shock, the 15-year-old patient exhibited a dramatic change in behaviour, at odds with his previous state, and accompanied by hypersomnia and confusion, megaphagia, irritability, hypersexuality and mood disorders. We observed a spontaneous and total regression of the symptoms after 12 days, except for the incomplete amnesia that proved to be persistent. Four months later, further to an ethylic drunkenness, the patient presented with a new and similar episode. The patient benefited from no medicinal treatment, even in the course of hypersomnia episodes and asymptomatic periods. After a clinical presentation of this patient, we will consider this case study from a more psychopathological angle by questioning the existence of a facilitating psychological profile. The discovery of an IQ equal to 86 from the scores of WISC-IV, and the identification of constructive visual difficulties made us suspect neurological disorders, but these abnormalities were not found during the completion of the Rey Complex Figure Test. The personality profile issued from the scores at the MMPI-A assessment was ranked as barely significant (type 2-4): indeed, it showed nothing specific to this patient. Literature data show that most of the patients presenting with a KLS have been seen by a psychiatrist at the time of the disease and diagnosed as suffering from hysteria, or schizophrenia, or bipolar disorders… Because of diagnostic wanderings, some patients have, hence, received inappropriate treatments. One should pay close attention to this very rare syndrome, on the border between neurology and psychiatry, since its diagnosis is essentially based on clinical features, and carefully think about the implementation of a medicinal treatment. This unique case seems unable to support our working hypothesis about the identification of a particular psychological profile in the KLS, but the question of an underlying fragility is still worth considering. We personally think that, even though links between the KLS and bipolar disorders have been suggested, this disease has to be considered as a separate entity.
Copyright (c) 2009 L’Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.
University Department of Psychiatry, Manchester Royal Infirmary.
Abstract
A 43-year-old man presented with an 18-month history of acute-onset cyclical behavioural change affecting mood, appetite, sleep, and energy levels. This had followed an initial episode of transient drowsiness which lasted 24 hours. On examination, there was some evidence of visual memory and frontal lobe deficits. A brain CT scan showed bilateral thalamic infarcts and a brain SPECT scan showed bilateral hypoperfusion of the frontal lobes. To our knowledge, this is the first reported case of thalamic infarction associated with acute-onset cyclical affective psychosis with clinical and neurophysiological features of frontal lobe syndrome. The case also highlights the possible role of thalamo-frontal circuits in the pathogenesis of the Kleine-Levin syndrome.
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