Sindrome de kleine levin pdf free

Kleine first described 2 cases of recurrent hypersomnia, hyperphagia, and cognitive disturbance in. Kleinelevin syndrome is rare, affecting an estimated 15 per million individuals. Kleinelevin syndrome kls is a rare sleep disorder characterized by persistent episodic hypersomnia and cognitive or mood changes. Kleine levin syndrome is a complex medical disorder that causes recurring periods of excessive drowsiness and sleep up to 20 hours per day. Abstract kleine levin syndrome kls is a rare disorder with symptoms that include periodic hypersomnia, cognitive and behavioural disturbances. Kleine levin syndrome is rare, affecting an estimated 15 per million individuals. Table 1 diagnostic criteria for kleinelevin syndrome as defined by. Therapeutic and diagnostic studies have the lowest evidence level. Full text full text is available as a scanned copy of the original print version. For language access assistance, contact the ncats public information officer. Gerardi, ma,1joseph casadonte, md,2,3 priyal patel, ba,1 and tanya k.

Nature and science of sleep dovepress kls foundation. Kleine levin syndrome kls is a rare disorder of unknown etiology. Pdf on dec 1, 2015, pedro ernesto barbosa pinheiro and others published. Kls is a rare disorder characterised by recurrent bouts of excessive sleep associated with reduced understanding of the world and altered behaviour. Kleinelevin syndrome kls is a rare sleep disorder mainly affecting. It is a disease predominantly of teenagers, and boys are four times more likely to be affected than girls. Awareness of kleine levin syndrome kls is very low. Many patients also experience hyperphagia, hypersexuality and other symptoms. A rare condition that is characterized by periods of excessive drowsiness and sleep, lasting up to 20 hours per day. Kleine levin syndrome foundation genetic and rare diseases. Um disturbio frequentemente associado a sintomas psicoticos, com hipersonia recorrente, bulimia e hipercinesia. Kleinelevin syndrome is a complex medical disorder that causes recurring periods of excessive drowsiness and sleep up to 20 hours per day.

Kleinelevin syndrome kleinelevin syndrome treatment. Individuals with jarcholevin typically appear to have a short trunk and neck, with arms appearing. Kleinelevin syndrome kls is a rare sleep disorder mainly affecting teenage boys in which the main features are intermittent hypersomnolence, behavioral and cognitive disturbances, hyperphagia, and in some cases hypersexuality. Kleine levin syndrome kls is a rare and complex neurological disorder characterized by recurring periods of excessive amounts of sleep, altered behavior, and a reduced understanding of the world. In dsmiv 9 the condition is covered by dyssomnianos while astheicdi0 10 classifies this condition in the chapter vi in diseases ofthe nervous system. Patients generally experience recurrent episodes of the condition for more than a decade and may return at a later age. These serious episodes alternate with periods without any symptoms. Kleinelevin hibernation syndrome, familial kleinelevin syndrome, familial hibernation syndrome. Electrophysiological changes during episodes of the kleine. Get a printable copy pdf file of the complete article 828k, or click on a page image below to browse page by page. Some patients have hallucinations or sexual disinhibition. This does not suggest that cases not included here do not represent kls. The relationship between the two syndromes, the praderwilli syndrome and the kleinesyndrome, deserves further study.

Kleine levin syndrome is a rare disorder that primarily affects adolescent males approximately 70 percent of those with kleine levin syndrome are male. Kleine levin syndrome is characterized by relapsingremitting episodes of severe hypersomnia, cognitive impairment, apathy, derealization and behavioural disturbances. Pathophysiologic hypotheses include a hypothalamic dysfunction and abnormalities in the central serotonin and dopamine metabolism. Kleinelevin syndrome is rare sleep disorder characterized by episodic hypersomnia, cognitive dysfunction, psychiatric and behavioral disturbances. Kleinelevin syndrome kls is a rare sleep disorder characterized by recurrent and unusually long episodes of hypersomnia, associated with behavioralcognitive disturbances, megaphagia and more rarely sexual disinhibition. Kleinelevin syndrome information page national institute. Case report refractory kleinelevin jscimed central. Kls support uk helping families affected by kleinelevin. Between episodes, patients have normal sleep, mood and behaviour. Kleinelevin syndrome was first described by kleine in 1925 and elaborated on by levin in 1936, but it was critchley and hoffman who further defined the syndrome in 1942 and gave it its eponym. This study is a retrospective study including 15 kls patients of the past 19 years.

It is characterized by recurring but reversible periods of excessive sleep up to 20 hours per day. Stimulants marginally address sleepiness, but may increase irritability and do not improve cognitive and behavioral disturbances. It is characterized by widespread and sometimes severe malformations of the vertebral column and ribs, shortened thorax, and moderate to severe scoliosis and kyphosis. Spondylocostal dysostosis, also known as jarcho levin syndrome jls, is a rare, heritable axial skeleton growth disorder. Links to pubmed are also available for selected references. Introduction kleine levin syndrome kls is a rare sleep disorder that affects 1 person in a million and has been suggested to be more frequent in ashkenazi jewish. Disostosis espondilotoracica genetic and rare diseases. Misdiagnosing sleep disorders as primary psychiatric. K leine levin syndrome kls is a sleep disorder of unknown etiology characterized by episodic hypersomnia, hyperphagia, cognitive and mood abnormalities, and abnormal behaviors. Kleinelevin syndrome is a recurrent hypersomnia associated with symptoms of hyperphagia, hypersexuality, and cognitive impairment. Spondylocostal dysostosis, also known as jarcholevin syndrome jls, is a rare, heritable axial skeleton growth disorder. Kleine levin syndrome rare neurological disorder duration. Most of the published studies have included only small numbers of patients.

In the light of this finding the autonomy of the syndrome seems questionable, since it differs only. Symptoms occur as episodes, typically lasting a few days to a few weeks. Symptoms may last for days to weeks, include excessive food intake, lack of energy, disorientation, irritability, and sensitivity to noise. Livia regina theilacker a, fabiola sampaio brandao a, fernanda velloso goulart a, joao luiz pereira vaz b, luiz octavio dias dalmeida a, maria cecilia da fonseca salgado a,b. Affected individuals usually have excessive food intake and may be confused, irritable, and lethargic. Functional imaging studies performed in small series of patients with kleine levin syndrome with visual or semiquantitative, uncontrolled analysis yielded. This site is like a library, you could find million book here by using search box in the. Jan 20, 2014 kleine levin syndrome is a recurrent hypersomnia associated with symptoms of hyperphagia, hypersexuality, and cognitive impairment. International classification of sleep disorders, 2nd edition. Pdf kleinelevin syndrome kls is the commonest recurrent sleep disorder with a. First ct findings in the kleinelevincritchley syndrome. Kleinelevin syndrome kls is a rare disorder of unknown etiology.

Recruitment in such families should favour multiplex families over single isolated cases. Kleinelevin syndrome kls is a rare disorder with symptoms that include periodic hypersomnia, cognitive and behavioural disturbances. If you have problems viewing pdf files, download the latest version of adobe reader. Citescore values are based on citation counts in a given year e. Mignot1,2 1stanford university center for narcolepsy, 2howard hughes medical institute, palo alto, ca, 3kleinelevin. One of the ways you can help change that is by printing out the kls brochure and sharing it with your community, friends, family, and health care professionals. Kleine levin syndrome and other inherited sleep disorders exclusion criteria 29583 no structural or inflammatory mslike lesions on brain mri. Kleine levin syndrome is an infrequent disorder of uncertain aetiopathogenesis that usually affects adolescent males, producing drowsiness and alterations in behaviour, appetite and sexuality. A case of a 19yearold man with a oneyear history of attacks of hypersomnia lasting several hours or days fitted the description of the kleinelevincritchley sindrome. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis.

The disease predominantly affects adolescent males. It is rare for patients over the age of 30 years to present with their first. All books are in clear copy here, and all files are secure so dont worry about it. Functional imaging studies performed in small series of patients with kleinelevin syndrome with visual or semiquantitative, uncontrolled analysis yielded. The full text of this article is available in pdf format.

Introduction kleinelevin syndrome kls is a rare sleep disorder that affects 1 person in a million and has been suggested to be more frequent in ashkenazi jewish. Kleinelevin syndrome kls is rare, but is still the most common form of recurrent hypersomnia. Most patients with kleinelevin syndrome are adolescent males. Abstract a case of kleinelevin syndrome, with chronic severe periodic hypersomnia is described in a 17yearold female. Kleinelevin syndrome is a rare disorder that primarily affects adolescent males approximately 70 percent of those with kleinelevin syndrome are male. Scribd is the worlds largest social reading and publishing site.

Stanford university center for narcolepsy, palo alto, ca, usa. If patients do experience psychotic symptoms such as delusions or hallucinations, they are shortlived and parallel the duration of the. Kleinelevin syndrome kls is a rare disease characterized by recurrent episodes of hypersomnia and to various degrees, behavioral or cognitive disturbances, compulsive eating behavior, and hypersexuality. The disorder strikes adolescents primarily but can occur in younger children and adults. Kleinelevin syndrome kls is a rare sleep disorder characterized by periodic. Division of neurology, hospital sao lucas da pontificia universidade. These singleton recruits will not contribute to the overall singleton monitoring metrics applied to gmcs. Ct scanning showed a hypodense area consisting of ectopic tissue of lipid consistency lying in the interpeduncular cistern.