Kleine-Levin Presentation

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Published on October 10, 2012

Author: mojoburrito

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Kleine-Levin Syndrome: Kleine-Levin Syndrome By: Molly Huisman What is Kleine-Levin Syndrome?: What is Kleine-Levin Syndrome? Kleine-Levin syndrome (KLS) is a periodic hypersomnia characterized by recurrent episodes of hypersomnia and at least one of the following symptoms: (1) cognitive or mood disturbances (2) megaphagia with compulsive eating (3) hypersexuality with inappropriate behaviors (4) abnormal behavior 1 Age of Manifestation of Symptoms: Age of Manifestation of Symptoms Most commonly, the syndrome involves teenagers and significantly disturbs the daily life of families, affecting schooling and any planned activities, but it can persist through adulthood and some adult onset cases have been reported. 1 Signs and Symptoms: Signs and Symptoms Symptoms occur as "episodes," typically lasting a few days to a few weeks Excessive food intake, irritability, childishness, disorientation, hallucinations, and an abnormally uninhibited sex drive may be observed during episodes. Mood can be depressed as a consequence, but not a cause, of the disorder Affected individuals are completely normal between episodes 3 Interesting Observations: Interesting Observations Episode onset is often abrupt, and may be associated with flu-like symptoms. Symptoms may be related to malfunction of the hypothalamus and thalamus, parts of the brain that govern appetite and sleep 3 Ways of Evaluating the Disease Process: Ways of Evaluating the Disease Process No specific marker for KLS is yet available. There is still no clue as to the origin of the disease. Cerebral fluid analysis is within normal limits, including level measurements of hypocretin-1, a hypothalamic peptide that has been shown to be deficient in the narcolepsy syndrome. Many neurologic tests have been normal such as electro-encephalogram, eliminating seizure disorder as a possibility. 1 Ways of Evaluating the Disease Process: Ways of Evaluating the Disease Process Sleep studies have also been conducted without results of high significance The results of brain imaging using CT scan and simple MRI were normal in all the well-documented cases of KLS The most interesting findings have come from at single-photon emission computed tomography (SPECT) analysis. Subsequently, it has been confirmed that the consistent finding during the symptomatic period is that of thalamic hypoperfusion , which is not seen during the asymptomatic period 1 Genetics of the Disease: Genetics of the Disease An immune-related dysfunction may be a cause in the symptomatic recurrence of symptoms of KLS patients. In addition, the by-product of a mild infection, such as fever, or the sleep fragmentation or deprivation, may modify the permeability of the blood-brain barrier. In association with a certain genetic background, this may lead to recurrence of symptoms for a short period, but there is no definitive proof to date. 1 Expected Outcomes: Expected Outcomes Episodes eventually decrease in frequency and intensity over the course of 8-12 years. 3 Nursing Implications: Nursing Implications There is no definitive treatment for Kleine-Levin syndrome and watchful waiting at home, rather than pharmacotherapy, is most often advised. Stimulant pills, including amphetamines, methylphenidate, and modafinil , are used to treat sleepiness but may increase irritability and will not improve cognitive abnormalities. Because of similarities between Kleine-Levin syndrome and certain mood disorders, lithium and carbamazepine may be prescribed and, in some cases, have been shown to prevent further episodes. 3 Nursing Implications: Nursing Implications Education may include eating healthy meals while awake Smoothies may be a good option as they provide the nutrients of food but also fluid intake Families need support Louisa Ball: Louisa Ball http://abcnews.go.com/Health/sleeping-beauty-kleine-levin-syndrome-makes-british-woman/story?id=13948399#.UHTVny5hS8A What’s Happening?: What’s Happening? The Stanford University Center for Narcolepsy has launched a study in January 2005 on Kleine-Levin Syndrome Kleine-Levin Syndrome Foundation http://klsfoundation.org/ References: References 1. Huang, Y., Lakkis , C., & Guilleminault , C. (2010). Kleine- levin syndrome: current status. Medical Clinics Of North America , 94 (3), 557-562. doi:10.1016/j.mcna.2010.02.011 2. Mignot E., Lammers G.J., Ripley B., et al:  The role of cerebro -spinal hypocretin measurement in the diagnostic of narcolepsy and other hypersomnias . Arch Neurol 59. 1255-1262.2002 References : References 3. Kleine-Levin Syndrome Information Page: National Institute of Neurological Disorders and Stroke (NINDS). (2009, March 12). National Institute of Neurological Disorders and Stroke (NINDS) . Retrieved October 9, 2012, from http://www.ninds.nih.gov/disorders/kleine_

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