Myotonic dystrophy type 2
Other Names for this Disease
- Dystrophia myotonica type 2
- Proximal myotonic myopathy
- Myotonic myopathy, proximal
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Your QuestionI'm 53 years old and have a very unusual sickness. Since I was 16 years old, I have been sick for several periods of the year. I was diagnosed with Kleine Levin syndrome four years ago, and the symptoms are very much like what other patients have described. Is there anyone else that has been sick with Kleine Levin syndrome for so long? I also received the diagnosis of myotonic dystrophy type 2 five years ago. Can Kleine Levin syndrome and myotonic dystrophy type 2 have anything to do with each other?
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Questions on this page
Kleine Levin syndrome is a rare disorder characterized by episodes of excessive sleep. Affected individuals may sleep for up to 20 hours per day during an episode. These episodes usually last for a few days to a few weeks. An episode may start abruptly and is sometimes associated with flu-like symptoms. During an episode, people with Kleine Levin syndrome can also display abnormal behavior, such as excessive food intake, irritability, childishness, disorientation, hallucinations, and an abnormally uninhibited sex drive. Affected individuals do not experience any of these features between episodes, and they may not be able to remember everything that happened during an episode. The time between episodes varies among individuals with this condition. Kleine Levin syndrome primarily affects adolescent males, but it also affects females. It may be caused by abnormal function of the hypothalamus and thalamus, parts of the brain that control appetite and sleep. Episodes usually decrease in frequency and intensity after about eight to 12 years.
Last updated: 3/2/2010
In most cases, the signs and symptoms of Kleine Levin syndrome last for about 8 to 12 years. In a review of 186 cases published in the medical literature, the authors found that the duration of Kleine Levin syndrome ranged from 6 months to 41 years.
Last updated: 3/2/2010
Myotonic dystrophy type 2, one of the two types of myotonic dystrophy, is an inherited muscular dystrophy that affects the muscles and other body systems (e.g., heart, eyes, and pancreas). It is characterized by prolonged muscle tensing (myotonia) as well as muscle weakness, pain, and stiffness. Signs and symptoms usually develop during a person's twenties or thirties. Muscles in the neck, fingers, elbows, and hips are typically affected; facial and ankle muscles are less commonly involved. The severity of myotonic dystrophy type 2 varies widely among affected people, even among family members. It is inherited in an autosomal dominant pattern and is caused by mutations in the CNBP gene. Treatment is based on each person's specific signs and symptoms.
Last updated: 2/10/2014
After an extensive search of the resources available to us, we were unable to find a connection between Kleine Levin syndrome and myotonic dystrophy type 2. We did find that people with myotonic dystrophy type 1 can have sleep disorders, but sleep disorders are not a feature of myotonic dystrophy type 2.
Last updated: 3/2/2010
- NINDS Kleine-Levin Syndrome Information Page. National Institute of Neurological Disorders and Stroke. March 12, 2009; http://www.ninds.nih.gov/disorders/kleine_levin/kleine_levin.htm. Accessed 3/2/2010.
- Arnulf I, Zeitzer JM, File J, Farber N, Mignot E. Kleine-Levin syndrome: a systematic review of 186 cases in the literature. Brain. 2005; 128(Pt12):2763-76. http://brain.oxfordjournals.org/cgi/content/full/128/12/2763. Accessed 3/1/2010.
- Dalton JC, Ranum LPW, Day JW. Myotonic Dystrophy Type 2. GeneReviews. April 23, 2007; http://www.ncbi.nlm.nih.gov/books/NBK1466/. Accessed 4/8/2012.
- Learning About Myotonic Dystrophy. National Human Genome Research Institute (NHGRI). June 4, 2012; http://www.genome.gov/25521207. Accessed 4/22/2015.
- Bouhour F, Bost M, and Vial C. Steinert Myotonic Dystrophy. Orphanet. April 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=273. Accessed 3/1/2010.
- Bouhour F. Proximal Myotonic Myopathy. Orphanet. July 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=606. Accessed 3/1/2010.