Battens'+Disorder

Oscar Bezi
//Batten's Disorder// //This disease is known as Batten's Disorder. It is the most common of a group of illnesses known as NCLS. Some other names for this disorder are Batten's Neuronal Ceroid Lipofuscinoses (NCL); Spielmeyer-Vogt-Sjoren-Batten's disease; NCL3; ceroid lipofuscinoses, neuronal 3, juvenile.// //Batten's Disorder is linked to an error in a gene known as CLN3, in chromosome 7. It is autosomal recessive, and can also be caused by a chromosoal error. Both parents need to have the gene error for it to be expressed in the child.//
 * //**Introduction**//
 * **//Mode of Inheritance//**



//The disease begins at age 6-12 when a previously normal infant begins having vision problems and seizures. Some victims also suffer from personality and behavior changes, slow learning, clumsiness, and stumbling. Then follows increasing mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually victims become blind, demented, bedridden, and suffer almost constant seizures if unmedicated. Batten's disease is fatal by the late teens or early twenties. It is more common in FInland, Sweden, and Northern Europe, as well as Newfoundland, Canada. In the UNited States, 2 to 4 out of 100,000 live births will have Batten's disease, which makes this a very rare disease according to the NIH, with only 0.004% risk of contraction. Batten's disease is caused be a buildup of lipofuscins, a type of lipid, in the nervous system, hence neuronal ceroid lipofuscinoses.// //As of now, no specific treatment can cure Batten's Disorder, nor halt/reverse the symptoms. Anticonvulsants, as well as pysical or occupational therapy can offer a temporary reprieve. To date there are no documented cases of a child surviving past the age of 25. There are no gene therapies available for curing Batten's disease.// //It is usually not detected until symptoms start. Many major universities are trying to make a prenatal test to determine whether or not a child has Batten's disorder. Since no definitive link has been made with gene CLN3, and since it is still impossible to tell if the gene error is for Batten's diesase and not another NCL, it is in fact diffiult to diagnose once it starts.//
 * **//Clinical Description//**
 * **//Treatment//**
 * **//Detection//**

CITING

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 * "Batten's Disease Fact Sheet." __National Institution of Neurological Disorders and Stroke__. 23 October 2009. National Institutes of Health. 12 April 2010.
 * "Human Karyotype." Karyotype. __Genome News Network__. 15 January 2003. 22 April 2010.
 * Kniffin, Cassandra L. "CLN3 GENE;CLN3." __Online Mendelian Inheritance in Man (OMIM)__. 10 August 2009. John Hopkins University. 12 April 2010.
 * "Treatments for Batten's Disease." __WrongDiagnosis.com__. 7 April 2010. 14 April 2010.