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Batten disease

Batten disease is a rare, fatal, inherited disease of the nervous system (neurodegenerative disorder) that begins in childhood. Early symptoms of this disorder usually appear between the ages of 5 and 10, when parents or physicians may notice a previously normal child has begun to develop vision problems or seizures. In some cases the early signs are subtle, taking the form of personality and behavior changes, slow learning, clumsiness, or stumbling.Other symptoms or signs include slowing head growth in the infantile form, poor circulation in lower extremities with legs and feet, decreased body fat and muscle mass, curvature of the spine, hyperventilation and/or breath-holding spells, teeth grinding, and constipation. more...

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Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten disease become blind, bedridden, and demented. Batten disease is often fatal by the late teens or twenties.

Batten disease is named after the British pediatrician F. E. Batten who first described it in 1903. Also known as Spielmeyer-Vogt-Sjogren-Batten disease, it is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs). Although Batten disease is usually regarded as the juvenile form of NCL, some physicians use the term Batten disease to describe all forms of NCL.

The disease is inherited in an autosomal recessive manner. The mutation causes the buildup of lipofuscins in the body's tissues. These substances consist of fats and proteins and form certain distinctive deposits that cause the symptoms and can be seen under an electron microscope. The diagnosis of Batten disease is based on the presence of these deposits in skin samples as well as other criteria. Six genes have now been identified that cause differenty types of Batten disease in children or adults. There are more that have yet to be identified. Two of these genes encode enzymes. The function of most of these geens is still unknown. The identification of these genes opens up the possibility of gene replacement therapy or other gene-related treatments.

In October 2005, the FDA approved the transplantation of fetal neuronal cells into the brains of children suffering from Infantile and Late Infantile versions of Batten disease. The cells, which are immature and in an early stage of development, are derived from aborted and miscarried fetuses and will be injected into the patient's brains. To avoid rejection of these foreign cells, the immune system of the patients has to be suppressed.

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The Neuronal Ceroid Lipofuscinoses (Batten Disease) (Biomedical and Health Research) $57.00 The Official Parent's Sourcebook on Batten Disease: A Revised and Updated Directory for the Internet Age $28.95
The dissection of a degenerative disease: Proceedings of four round-table conferences on the pathogenesis of Batten's disease (neuronal ceroid-lipofuscinosis) $190.92 Cystic Fibrosis $24.18
Batten Disease: Diagnosis, Treatment, Research (Advances in Genetics, Vol 45) (Advances in Genetics) $64.23 Batten Disease: A Medical Dictionary, Bibliography, And Annotated Research Guide To Internet References $32.71
Ceroid Liposfucsinosis: Batten's Disease $132.00 Batten disease (SuDoc HE 20.3502:B 32)
Ceroid-lipofuscinosis (Batten's disease): Proceedings of the International Symposium on Human and Animal Models of Ceroid-lipofuscinosis, June 1 & 4, 1980, RĀ²ros, Norway Opitz: Ceroid-Lipofuscinoses- Batten Disease & Allie Disorders (Proc Staten Island NY 1987) $94.50

StemCells, Inc. Receives FDA Clearance to Initiate Phase I Clinical Trial of Neural Stem Cells to Treat Batten Disease; First-Ever FDA-Approved Trial to
PALO ALTO, Calif. -- StemCells, Inc. (NASDAQ: STEM) today announced that it has received clearance from the U.S. Food and Drug Administration (FDA) to begin a Phase I safety and preliminary efficac
StemCells, Inc. Announces Filing of IND Amendment for Human Neural Stem Cell Transplant Treatment for Batten Disease
PALO ALTO, Calif. -- StemCells, Inc. (NASDAQ: STEM) today announced the filing with the U.S. Food and Drug Administration (FDA) of an amendment to its ...
StemCells, Inc. Announces Filing of IND for Human Neural Stem Cell Transplant Treatment for Batten Disease
PALO ALTO, Calif. -- Phase I Clinical Trial at Stanford Would Be First-Ever Using Transplantation of Human Neural (Brain) Stem Cells StemCells, Inc.
Gene tied to childhood brain disorder - mutated gene on chromosome 16 identified as cause of Batten disease - Biology - Brief Article
A child between the ages of 5 and 10 years suddenly goes blind. Seizures occur with increasing frequency, and the child inexorably loses his or her mental ...
Pulmonary capillary hemangiomatosis: a consideration in unexplained pulmonary hypertension
INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare condition which causes severe pulmonary hypertensive disease. There are overlapping ...
Study Finds Autoimmune Link In Juvenile Batten Disease
For years, researchers have tried to determine how the defective gene in juvenile Batten disease leads to the seizures, mental impairment, and other symptoms of this devastating childhood disorder.
Critically III child has 'Hands On Racing' experience at Indy
Six-year-old Baltimore native Nicholas Heuchan, a pediatric patient battling juvenile Batten disease, a life-threatening illness that causes blindness ...
Only 500 children in the world suffer from a disease that will kill
TINA and Mark Harris have to live with the unbearable knowledge that both their children will die before the age of nine. Jordan, six, and Jasmine, ...

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