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Batten Disease
 Batten disease is a common form of a group of disorders called neuronal ceroid lipofuscinoses (or NCLs) . Over time affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills, and over time leading to blindness and being bedridden unable to communicate.  Juvenile NCL ( Batten Disease) begins between the ages of 5 and 8 years of age. Early signs are vision loss, seizures, ataxia, (loss of muscle coordination) or clumsiness.

Batten disease and other forms of NCL are relatively rare and often strike more than one person in the families that carry the defective genes.  When both parents carry one defective gene, each of their children faces one in four chance of developing NCL.

Symptoms of Batten Disease are linked to the buildup of substances called lipopigments in the bodies tissues. These lipopigments are made up of fats and proteins. They build up in the cells of the brain and the eye as well as in skin, muscle and many other tissues. These deposits are what doctors look for when the examines a skin sample to diagnose Batten Disease.

Diagnostic testing used for NCLs include: Blood and urine tests. Doctors looking for consisting elevated levels of a chemical called dolichol which is found in the urine of many NCL patients. Doctors also examine a small piece of tissue under the microscope looking for typical NCL deposits. An EEG (electrocencephalogram) uses  special patches placed on the scalp to record electrical currents inside the brain.  CT scans, of the brain’s tissues and structures. MRI (magnetic resonance imaging) uses a combination of magnetic fields and radio waves to create a picture of the brain.

Batten is not contagious or preventable at this time, and there is no specific treatment that can halt or reverse the symptoms of Batten Disease or other NCLs. However seizures can sometimes be reduced with anticonvulsant drugs, as well as occupational therapy helping patients retain functions as long as possible.  Their are four main forms of Batten Disease and for more information please contact the foundation listed below. Through public support and funding, a possible cure may come sometime in the future. Please help us eradicate all childhood diseases! Stop our children from suffering.  Support this worthwhile cause Please.

For more information please contact..

Brittany’s Friends Fund For Childhood
Brain Disease Research
Box 189
Ruthven, ON
NOP 2GO

britjar@wincom.net
Phone:
(519) 322-2987
Fax:
(519) 326-0434

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