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Topic: Batten disease

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	Batten Disease
		Batten disease is a fatal, inherited disorder of the nervous system that begins in childhood.
		Batten disease is often fatal by the late teens or twenties.
		Batten disease and other forms of NCL are relatively rare, occurring in an estimated 2 to 4 of every 100,000 live births in the United States.
	healthlink.mcw.edu /article/921388974.html (1802 words)

	NCL resource
		Batten disease is a group of related disorders which affects cells in the brain.
		Batten disease is also called 'neuronal ceroid lipofuscinosis' because there is a build-up of material in most cells that resembles substances called ceroid and lipofuscin, but it is the neuronal cells of the brain that are affected most and eventually die.
		Batten disease is an inherited or genetic disease.
	www.ucl.ac.uk /ncl/batten.shtml (589 words)

	Batten Disease
		Batten disease is an inherited disorder that leads to a progressive deterioration of the brain and nervous system, and inevitably leads to an early death.
		The development of Batten disease is connected to a buildup of lipopigments in body tissues, including cells of the brain, the eye, the skin, and muscles.
		Batten disease is often first suspected by an ophthalmologist because, except in the adult form, vision loss is one of the earliest symptoms.
	www.visionrx.com /library/enc/enc_batten.asp (670 words)

	Brain Foundation - Batten Disease
		Although Batten Disease is usually regarded as the juvenile form of NCL, it has now become the term to describe all forms of NCL.
		Juvenile Batten Disease is always fatal by the late teens or twenties.
		Batten Disease and other forms of NCL are relatively rare, occurring in an estimated 2 to 4 of every 100,000 births in the United States.
	www.brainaustralia.org.au /AZ_of_Brain_Disorders/batten_disease (848 words)

	Batten Disease
		Symptoms of Batten disease — a rare, progressive disorder of the nervous system — usually appear in children between the ages of 5 and 10.
		The early signs of Batten disease also can be subtle, and include personality and behavior changes, slow learning, clumsiness, or stumbling.
		Batten disease is inherited and there is no specific treatment to halt or reverse the symptoms.
	www.habibintl.com /batten_disease.htm (212 words)

	What is Batten Disease?
		Batten disease and other forms of NCL are relatively rare, occurring in an estimated 4 of every 100,000 births in the United States.
		The cause of Batten disease lies in the chromosomes, which carry the hereditary characteristics and are found in the nuclei of somatic cells.
		Batten disease is rarely diagnosed immediately because of the variability in symptoms and age of onset.
	www.battens.org.au /about.html (1974 words)

	Batten Disease - NYU Medical Center, NYU Hospital, New York, NY
		Batten disease is the most common form of a group of rare disorders known as neuronal ceroid lipofuscinoses (NCLs).
		Batten disease is an inherited genetic disorder that causes a build-up of lipopigments in the body’s tissue.
		Batten disease refers to the juvenile form of NCL, but the other forms of NCL can also be referred to as Batten disease.
	www.med.nyu.edu /patientcare/library/article.html?ChunkIID=23620 (760 words)

	BDSRA - Batten Disease Support and Research Association
		Batten Disease/NCL is relatively rare, occurring in an estimated 2 to 4 of every 100,000 births in the United States.
		In September 1995, The International Batten Disease Consortium announced the identificatiion of the gene for the juvenile form of Batten Disease.
		Cell lines of persons with Batten Disease and their families are grown and maintained in cell banks located at Institute for Basic Research in Staten Island, NY and at Massachusetts General Hospital.
	www.bdsra.org /batten.htm (2175 words)

	[No title]
		Batten disease tends to be slightly more common in Scandinavia and other parts of northern Europe as well as in the parts of American where those ehnic groups are well represented, for example in Minnesota and around Chicago.
		For families watching their children suffer from the illness, Batten disease is a devastating and often baffling diagnosis.
		The disorder’s rarity means that most doctors have never seen a case of Batten disease, and most families have never heard of it before their children are diagnosed.
	jscms.jrn.columbia.edu /cns/2005-04-19/russell-battendisease/fullText (857 words)

	Batten Disease
		Batten Disease is the juvenile form of a group of progressive neurological diseases called 'neuronal ceroid lipofuscinoses' (NCL).
		Researchers suspect that Batten Disease, and other forms of NCL, are caused by missing enzymes resulting in the body's inability to break down substances in the normal way...such as fat and associated sugars and proteins.
		Batten Disease leads to a vegetative state and is ultimately fatal.
	www.nataliefund.org /batten.html (498 words)

	025.431: The Dewey blog: Batten Disease
		Batten disease is mentioned and indexed in DDC at 616.83 Other organic diseases of central nervous system, but you might not immediately recognize it: “Including.
		Batten disease in children is classed in 618.9283 Other pediatric organic diseases of central nervous system (built with 618.92 Pediatrics plus 83 from 616.83 Other organic diseases of central nervous system according to instructions at 618.921-618.929 Specific pediatric diseases).
		Batten disease in dogs is classed in 636.7089683 Other canine organic diseases of central nervous system (built with 636.7 Dogs plus 0 from add instruction at 636.7001-636.708 Standard subdivisions, specific topics in husbandry of dogs plus 89 from 636.089 Veterinary sciences Veterinary medicine plus 683 from 616.83 Other organic diseases of central nervous system).
	ddc.typepad.com /025431/2006/06/batten_disease.html (584 words)

	Batten Disease Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)
		Because the mutated genes that are involved in certain forms of Batten disease are known, carrier detection is possible in some instances.
		Symptoms of Batten disease and other NCLs are linked to a buildup of substances called lipofuscins (lipopigments) in the body's tissues.
		In families where the mutation in the gene for CLN3 is known, DNA analysis can be used to confirm the diagnosis or for the prenatal diagnosis of this form of Batten disease.
	www.ninds.nih.gov /health_and_medical/pubs/batten_disease.htm (1821 words)

	Batten Disease - WrongDiagnosis.com
		Batten Disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
		This means that Batten Disease, or a subtype of Batten Disease, affects less than 200,000 people in the US population.
		Batten disease is a rare, fatal, inherited disease of the nervous system (neurodegenerative disorder) that begins in childhood.
	www.wrongdiagnosis.com /b/batten_disease/intro.htm (547 words)

	NPR : Batten Disease Unites Parents, Dog Owners
		By collaborating with the human Batten disease community, they're hoping not only to find a cure, but to have the tools necessary to test all dogs before they're bred.
		Erika Gaspar poses with her dog, Misha, who is showing a variety of symptoms that may indicate Batten disease.
		Batten disease is a rare inherited genetic disorder leading to a breakdown of the entire nervous system.
	www.npr.org /templates/story/story.php?storyId=5497047 (824 words)

	NIH Press Release - Gene for Last Major Form of Batten Disease Discovered - 09/18/1997
		Late infantile Batten disease, also called Jansky-Bielchowsky disease or late infantile neuronal ceroid lipofuscinosis (LINCL), is one of four closely related metabolic disorders that lead to neurodegeneration.
		Late infantile Batten disease affects about 300 children and accounts for about 40 percent of the NCL seen in the United States.
		The lipofuscin that accumulates within lysosomes in Batten disease is similar in some ways to the lipofuscin that accumulates in aged cells, says Dr. Pullarkat.
	www.nih.gov /news/pr/sept97/ninds-18.htm (1375 words)

	Family takes up cause of Batten disease
		Both parents are carriers of the gene for Batten disease, but only Zachery has developed it.
		Batten is one of 40 of what are called lysosomal storage disorders.
		In these disorders, including the more common Hunter's disease or Tay-Sachs syndrome, the enzymes that break down fats, proteins and sugars in cells are lacking, which gradually halts brain development.
	www.post-gazette.com /healthscience/20020910hbatten0910p4.asp (953 words)

	Illnesses and Disabilities - Health Problems & Types of Disabilities - Batten Disease
		Symptoms of Batten disease — a rare, progressive disorder of the nervous system — usually appear in children between the ages of 5 and 10.
		NINDS Batten Disease Fact Sheet - This publication discusses the prevalence, causes, diagnosis and treatment of Batten disease and contains information on current research being conducted.
		Batten Disease Facts and Fictions (Copyright © BDSRA) - This publication discusses the diagnosis and treatment of Batten Disease and contains information on current research being conducted.
	www.4woman.gov /wwd/conditions/batten.cfm?style=module (332 words)

	New center focuses on rare Batten disease (Site not responding. Last check: 2007-11-03)
		For children with Batten disease and their families, the cruelty of the disease, which robs children of their sight, their cognitive faculties and finally their lives, is made worse by the hunt for a doctor experienced at recognizing and treating patients.
		Since the disease affects only about 1,000 children in the United States, most doctors have never seen a child with the disease - for families that usually means a string of referrals from doctor to doctor who try to pinpoint the cause of the child's symptoms.
		The center's resources include genetic testing for the disease; visits with doctors who have seen dozens of children with the disorder; information on what families can expect as the disease progresses; and development of research tools to help scientists seek better treatments or a cure.
	www.news-medical.net /?id=8502 (996 words)

	Batten disease - Wikipedia, the free encyclopedia
		Batten disease is named after the British pediatrician Frederick Batten who first described it in 1903.
		The disease is inherited in an autosomal recessive manner.
		The diagnosis of Batten disease is based on the presence of these deposits in skin samples as well as other criteria.
	en.wikipedia.org /wiki/Batten_disease (592 words)

	Study finds autoimmune link in juvenile Batten disease
		Juvenile Batten disease is a fatal, inherited childhood neurodegenerative disorder that results from mutations in a gene called CLN3.
		The disease is usually fatal by the late teens or twenties.
		Juvenile Batten disease is the most common of a group of disorders called neuronal ceroid lipofuscinoses, or NCLs.
	www.eurekalert.org /pub_releases/2002-05/nion-sfa052202.php (1072 words)

	Batten Disease Symptom & Signs
		Symptoms of Batten disease are linked to a buildup of substances called lipopigments in the body's tissues.
		Diagnostic tests for Batten disease include blood or urine tests, skin or tissue sampling, an electroencephalogram (EEG), electrical studies of the eyes, and brain scans.
		As yet, no specific treatment is known that can halt or reverse the symptoms of Batten disease.
	www.habibintl.com /batten_disease_symptoms.htm (272 words)

	Signs of Batten Disease - WrongDiagnosis.com
		The phrase "signs of Batten Disease" should, strictly speaking, refer only to those signs and symptoms of Batten Disease that are not readily apparent to the patient.
		The word "symptoms of Batten Disease" is the more general meaning; see symptoms of Batten Disease.
		This medical information about signs and symptoms for Batten Disease has been gathered from various sources, may not be fully accurate, and may not be the full list of Batten Disease signs or Batten Disease symptoms.
	www.wrongdiagnosis.com /b/batten_disease/signs.htm (408 words)

	BATTEN DISEASE : Contact a Family - for families with disabled children: information on rare syndromes and disorders
		The group of diseases known as Batten disease or the neuronal ceroid lipofuscinoses (NCL) are progressive inherited metabolic diseases that occur in children and adults.
		Batten disease is relatively rare occurring in about 1 in 30,000 births.
		Although our understanding of Batten disease is improving all the time, there is at present no cure or treatment that has any significant impact on the inexorable decline in bodily functions and inevitable early death.
	www.cafamily.org.uk /Direct/b11.html (813 words)

	Pearce Lab- Batten Disease Research (Site not responding. Last check: 2007-11-03)
		Batten disease is a type of Neuronal Ceroid Lipofuscinosis, so called because of the fact that a part of the cell called the lysosome accumulates a heterogeneous mix of fat-like material called lipofuscin.
		Batten disease results from the inheritance of two mutated copies of the human CLN3 gene.
		Children with Batten disease slowly lose their vision from around the age of 5-7 years of age.
	dbb.urmc.rochester.edu /labs/pearce/index.htm (332 words)

	URMC Batten Disease and Clinical Research Center - Clinical Evaluation (Site not responding. Last check: 2007-11-03)
		Families should expect to receive a comprehensive evaluation for their child or children with Batten disease when they visit the Batten Disease Diagnostic and Clinical Research Center at the University of Rochester.
		The Batten Disease Center will offer neuropsychological and behavioral assessment to help clarify your child’s pattern of cognitive strengths and weaknesses, and develop recommendations for educational services and behavior management.
		Educational consultation is also available, including review of school records, providing schools with information on Batten Disease, and assisting schools with planning their own evaluations and services for the child with Batten’s.
	dbb.urmc.rochester.edu /labs/pearce/bddcrc/clinical_eval.htm (628 words)

	Batten Disease
		It is possible that the main title of the report Batten Disease is not the name you expected.
		Batten disease is the juvenile form of a group of progressive neurological diseases known as neuronal ceroid lipofuscinoses (NCL).
		This disease entry is based upon medical information available through the date at the end of the topic.
	www.webmd.com /hw/health_guide_atoz/nord259.asp (453 words)

	[No title]
		Each form of Batten disease is the result of inheriting two mutated copies of genetic material, ultimately causing the devastating effects of these disorders.
		Unfortunately the vast majority of these diseases are incurable with profound effects upon the brain and affected children suffer a long period of disability and a premature death.
		This is not only big news for Batten disease, but is the first clinical trial of this type of cell lines in any neurodegenerative disorder.
	www.iop.kcl.ac.uk /iopweb/departments/home/default.aspx?locator=382 (769 words)

	Batten Disease Information Page: National Institute of Neurological Disorders and Stroke (NINDS) (Site not responding. Last check: 2007-11-03)
		Batten Disease fact sheet compiled by NINDS, the National Institute of Neurological Disorders and Stroke.
		May 2002 press release on an autoimmune reaction in juvenile Batten disease.
		Información del enfermedad de Batten/Spanish-language fact sheet on Batten disease compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
	www.ninds.nih.gov /health_and_medical/disorders/batten.htm (586 words)

	The Luke & Rachel Batten Foundation (Site not responding. Last check: 2007-11-03)
		Late Infantile NCL (Jansky-ielschowsky disease) begins between ages 2 and 4.
		Juvenile NCL (Batten Disease) begins between the ages of 5 and 8 years of age.
		Batten Disease and other forms of NCL occurr in an estimated 2 to 4 of every 100,000 births in the United States.
	lrbf.org /WhatIsBatten.asp (838 words)

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