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Neuronal Ceroid Lipofuscinosis:     more detail

Lysosomal Storage Diseases: Tay-Sachs Disease, Canavan Disease, Sly Syndrome, Neuronal Ceroid Lipofuscinosis, Mucopolysaccharidosis The dissection of a degenerative disease: Proceedings of four round-table conferences on the pathogenesis of Batten's disease (neuronal ceroid-lipofuscinosis) Lipofuscin and Ceroid Pigments (Advances in Experimental Medicine and Biology) Batten Disease: Diagnosis, Treatment, and Research, Volume 45 (Advances in Genetics) The Official Parent's Sourcebook on Batten Disease: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2002-11-18 Lipofuscin and Ceroid Pigments: State of the Art 1995 (Journal - Gerontology, , Vol 41, Suppl. 2) (Pt.2) Dogs help track down genes.(MEDICAL UPDATE: Cutting-edge news from a source you can trust)(Batten disease): An article from: Saturday Evening Post Batten disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Michelle lee Brandt, Rosalyn, MD Carson-Dewitt, 2005 Batten disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Michelle Brandt, 2005 Batten disease (SuDoc HE 20.3502:B 32) by U.S. Dept of Health and Human Services, 1992

lists with details

81. Neuronal Ceroid Lipofuscinosis And Arthropathy: A Family Study -- Proops And Gre
    neuronal ceroid lipofuscinosis and arthropathy a family study. R Proops and SH Green. A family is described in which three children have neuronal ceroid  
    http://jmg.bmjjournals.com/cgi/content/abstract/18/2/101
    
    Extractions: Vol Page [Advanced] This Article Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Proops, R. Articles by Green, S. H. Journal of Medical Genetics, 1981, Vol 18, 101-104 R Proops and SH Green

82. Sara Mole
    Biology of the neuronal ceroid lipofuscinoses (Batten disease) Variant late infantile neuronal ceroid lipofuscinosis in a subset of Turkish patients is  
    http://www.ucl.ac.uk/LMCB/pages/mole.html
    
    Extractions: Email: s.mole@ucl.ac.uk Autofluorescent lipofuscin in patient cells (courtesy of Prof Hans Goebel) The neuronal ceroid lipofuscinoses (NCL), or Batten disease, are the most common neurodegenerative disorders of childhood. Children suffer from progressive blindness and neurodegeneration leading to premature death. The disease is characterised by the accumulation of autofluorescent material (ceroid and lipofuscin-like) in the lysosomes of most cells, and the death of cortical neurons. Since lipofuscin accumulates during the normal ageing process and ceroid is found in several diseases, including a common cause of blindness, and since the NCL storage material accumulates prenatally yet may have no clinical effects for many years, understanding the molecular basis of NCL disease may also shed light on the biology of ageing, macular degeneration and late onset neurodegeneration. Most NCLs are inherited in an autosomal recessive manner. I have spent many years taking a molecular genetic approach to identify genes defective in the different types of NCL. Six human genes have been identified so far, and one additional gene has been identified in a naturally occurring animal model. Four of the genes encode soluble proteins that are found in the lysosome (

83. Arch Ophthalmol -- Abstract: Diagnosis Of Neuronal Ceroid Lipofuscinosis By Ultr
    Diagnosis of neuronal ceroid lipofuscinosis by ultrastructural examination of peripheral blood lymphocytes. RD Brod, AJ Packer and HJ Van Dyk  
    http://archopht.ama-assn.org/cgi/content/abstract/105/10/1388
    
    Extractions: Vol. 105 No. 10, October 1987 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Brod RD Van Dyk HJ Contact me when this article is cited R. D. Brod, A. J. Packer and H. J. Van Dyk LSU Eye Center, Louisiana State University Medical Center School of Medicine, New Orleans 70112-2234. A 7-year-old child with a history of seizures, psychomotor regression, and progressive visual loss was found to have juvenile type neuronal ceroid lipofuscinosis on the basis of characteristic ophthalmoscopic and electroretinographic findings. Although transmission electron micrographs

84. Journal Of Heredity -- Sign In Page
    Characterization of Candidate Genes for neuronal ceroid lipofuscinosis in Dog J Hered Drögemüller et al. 10.1093/jhered/esi088  
    http://jhered.oxfordjournals.org/cgi/content/full/esi088/DC1
    
    Extractions: This item requires a subscription* to Journal of Heredity Online. * Please note that articles prior to 1996 are not normally available via a current subscription. In order to view content before this time, access to the Oxford Journals digital archive is required. Alternatively, you may purchase short-term access on a Pay per Article basis. Please see below for more details.

85. Deutsches Ärzteblatt: Archiv
    Translate this page Autosomal dominant adult neuronal ceroid lipofuscinosis a novel form of NCL with Variant late infantile neuronal ceroid lipofuscinosis in a subset of  
    http://www.aerzteblatt.de/v4/archiv/lit.asp?id=45242

86. AllRefer Health - Neuronal Ceroid Lipofuscinoses (NCLS) (Batten Disease, Jansky-
    neuronal ceroid Lipofuscinoses (NCLS) (Batten Disease, JanskyBielschowsky, Kufs Disease, Lipofuscinoses, Spielmeyer-Vogt) information center covers causes  
    http://health.allrefer.com/health/lipofuscinosis-info.html
    
    Extractions: AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia Go To Main Page Alternate Names : Batten Disease, Jansky-Bielschowsky, Kufs' Disease, Lipofuscinoses, Spielmeyer-Vogt Definition The neuronal ceroid lipofuscinoses (NCLS) are a group of rare, inherited neurodegenerative disorders. They associated with the accumulation of an abnormal pigment in the brain called lipofuscin. These disorders can be associated with severe diseases including blindness, mental retardation, and early death. There are three main types, depending on the age it begins late infantile (Jansky-Bielschowsky), juvenile (Batten disease), and adult (Kufs or Parry's disease).

87. AllRefer Health - Neuronal Ceroid Lipofuscinoses (NCLS): Symptoms & Signs (Batte
    neuronal ceroid Lipofuscinoses (NCLS) (Batten Disease, JanskyBielschowsky, Kufs Disease, Lipofuscinoses, Spielmeyer-Vogt) information center covers  
    http://health.allrefer.com/health/lipofuscinosis-symptoms.html
    
    Extractions: AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia

88. Neuronal Ceroid-Lipofuscinosis - Batten Disease - Information Page With HONselec
    An inherited degenerative disease characterized by neuronal cytoplasmic inclusio  
    http://www.hon.ch/HONselect/RareDiseases/EN/C10.574.500.550.html
    
    Extractions: Neuronal Ceroid-Lipofuscinosis Definition: An inherited degenerative disease characterized by neuronal cytoplasmic inclusions which stain positively for ceroid and lipofuscin. Affected individuals develop retinal degeneration, seizures, myoclonus, ataxia, rigidity, and progressive dementia. Clinically there are four subtypes, divided by age of onset of symptoms: infantile (Santavuori-Haltia type), late infantile (Jansky-Bielschowsky type), juvenile (Spielmeyer-Vogt type), and adult (Kuf's disease). The late infantile and juvenile forms may both also be referred to as Batten Disease and Batten-Mayou Disease. (Adams et al., Principles of Neurology, 6th ed, p957)

89. HONselect - Neuronal Ceroid-Lipofuscinosis
    Translate this page English, neuronal ceroid-lipofuscinosis, - Batten Disease - ceroid-lipofuscinosis, neuronal - Jansky-Bielschowsky Disease - Kufs Disease  
    http://www.hon.ch/HONselect/RareDiseases/C10.574.500.550.html

90. Neuronal Ceroid-Lipofuscinosis
    neuronal ceroidlipofuscinosis This resource has a US focus. Patient Education Handout Publication Type; neuronal ceroid-lipofuscinosis;  
    http://omni.ac.uk/browse/mesh/D009472.html
    
    Extractions: low graphics NINDS : Batten disease information page This Web resource on Batten disease (a fatal, inherited disorder of the nervous system that begins in childhood) is produced by the National Institute of Neurological Disorders and Stroke (NINDS). A description of Batten disease is provided, and available treatments, prognosis, and current research activities are all discussed. Links to related organisations and NINDS related material (including documents and press releases) are provided. This resource has a US focus. Patient Education Handout [Publication Type] Neuronal Ceroid-Lipofuscinosis

91. Short Description Of Cell Lines. Pathology: Neuronal Ceroid-lipofuscinosis Infan
    Version 4.200205. Short description of cell lines. Pathology neuronal ceroidlipofuscinosis infantile Finnish type 256730 OMIM record  
    http://www.biotech.ist.unige.it/cldb/pat134.html

92. Neuronal Ceroid-lipofuscinosis - Definition From Biology-Online.org
    Definition and other additional information on neuronal ceroidlipofuscinosis from Biology-Online.org dictionary.  
    http://www.biology-online.org/dictionary/neuronal_ceroid-lipofuscinosis
    
    Extractions: Directory ... N neuronal ceroid-lipofuscinosis A heritable lipidosis with cytoplasmic inclusions staining for ceroid and lipofuscin . Clinically the patient has progressive dementia retinal degeneration seizures , and myoclonic jerks . It was formerly thought to be related to tay-sachs disease but the biochemical defect is as yet undetermined. Named according to age of onset : jansky-bielschowsky disease years batten spielmeyer-vogt disease , 5-11 years; and kufs disease adult

93. GeneReviews: Neuronal Ceroid-Lipofuscinosis
    Your browser does not support HTML frames so you must view neuronal ceroidlipofuscinosis in a slightly less readable form. Please follow this link to do  
    http://www.geneclinics.org/profiles/ncl/?Lng=GB

94. Neuronal Ceroid-lipofuscinosis
    neuronal ceroidlipofuscinosis. ceroid lipofuscinosis Synapse Web - Medical College of Georgia. Search with keyword(s) And Or Wildcard = *  
    http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=526

95. MeSH-D Terms Associated To MeSH-C Term Neuronal Ceroid-Lipofuscinosis
    MeSHD terms associated to MeSH-C term neuronal ceroid-lipofuscinosis, G2D Home association of the corresponding term to neuronal ceroid-lipofuscinosis.  
    http://www.bork.embl-heidelberg.de/g2d/c2d.pl?Neuronal_Ceroid-Lipofuscinosis:unk

96. Dorlands Medical Dictionary
    ceroidlipofuscinosis, neuronal ceroid-lipofuscinosis, a term for several genetic lipidoses of diverse biochemical and clinical characteristics,  
    http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

97. Atypical Neuronal Ceroid-lipofuscinosis -- Greenwood And Nelson 28 (7): 710 -- N
    were similar to those in typical cases of neuronal ceroidlipofuscinosis. Atypical neuronal ceroid-lipofuscinosis may be distinguished histochemically,  
    http://www.neurology.org/cgi/content/abstract/28/7/710
    
    Extractions: Submit a response Alert me when this article is cited Alert me when Correspondence are posted ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Greenwood, R. S. Articles by Nelson, J. S. RS Greenwood and JS Nelson We describe the clinical, pathologic, and ultrastructural findings in a case of juvenile onset neuronal ceroid-lipofuscinosis without visual symptoms or retinal abnormalities. The histochemical and ultrastructural characteristics of the neuronal lipopigment were similar to those in typical cases of neuronal ceroid-lipofuscinosis. Atypical neuronal ceroid-lipofuscinosis may be distinguished histochemically, ultrastructurally, and clinically from another disorder called juvenile

98. Juvenile-onset Neuronal Ceroid-lipofuscinosis In Rambouillet Sheep -- Edwards Et
    Juvenileonset neuronal ceroid-lipofuscinosis in Rambouillet sheep. JF Edwards, RW Storts, JR Joyce, JM Shelton and CS Menzies  
    http://www.vetpathology.org/cgi/content/abstract/31/1/48
    
    Extractions: HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS This Article Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager PubMed PubMed Citation Articles by Edwards, J. F. Articles by Menzies, C. S. J. F. Edwards, R. W. Storts, J. R. Joyce, J. M. Shelton and C. S. Menzies Two, 8-month-old Rambouillet half-sister ewes with signs of visual loss and decreased mentation were examined. Ewe No. 1 was necropsied at 10 months of age, and after being held under observation for a further 6 months, ewe No. 2 was necropsied at 16 months of age. At that time, the ewe was blind and severely depressed. Both ewes had deposition of an autofluorescent lipopigment, identified as ceroid-lipofuscin, in neurons of the brain, spinal cord, eye, and dorsal root ganglia. The disease process was progressive and characterized by deposition of lipopigment with neuronal degeneration and severe fibrillary astrogliosis. This progressive loss of

99. Neuronal Ceroid-lipofuscinosis In A Cat -- Bildfell Et Al. 32 (5): 485 -- Veteri
    neuronal ceroidlipofuscinosis was diagnosed in a young adult domestic Contrary to the human neuronal ceroid-lipofuscinoses, pigment deposition appeared  
    http://www.vetpathology.org/cgi/content/abstract/32/5/485
    
    Extractions: HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS This Article Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager ... Cited by other online articles PubMed PubMed Citation Articles by Bildfell, R. Articles by Ward, P. R. Bildfell, C. Matwichuk, S. Mitchell and P. Ward Department of Pathology and Microbiology, Atlantic Veterinary College, University of Prince Edward Island, Canada. Neuronal ceroid-lipofuscinosis was diagnosed in a young adult domestic short-haired cat euthanatized because of severe progressive neurologic disease. Clinical signs included blindness, seizures, and decreased mentation. An autofluorescent pigment, identified as ceroid-lipofuscin by electron microscopy and staining properties, was found within neurons of the central and peripheral nervous systems. A diffuse reactive astrocytosis accompanied by multifocal microgliosis was visible in all areas of the brain. Retinal atrophy with intraneuronal lipopigment accumulation was also

100. EMedicine - Neuronal Ceroid Lipofuscinoses : Article By Celia H Chang, MD
     neuronal ceroid Lipofuscinoses The neuronal ceroid lipofuscinoses (NCLs), also known as Batten disease, are a group of neurodegenerative disorders.  
     http://www.emedicine.com/neuro/topic498.htm
     
     Extractions: (advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Neurology Pediatric Neurology Last Updated: February 20, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography Author: Celia H Chang, MD , Assistant Professor, Department of Neurology, University of California at Davis Celia H Chang, MD, is a member of the following medical societies: American Academy of Neurology , and Child Neurology Society Editor(s): Beth A Pletcher, MD , Co-Director of The Neurofibromatosis Center of New Jersey, Associate Professor, Department of Pediatrics, University of Medicine and Dentistry of New Jersey; Francisco Talavera, PharmD, PhD , Senior Pharmacy Editor, eMedicine; Kenneth J Mack, MD, PhD , Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic;

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