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         Progressive Infantile Poliodystrophy:     more detail

61. NINDS - Neurological Disorder Information
Leukoencephalopathy progressive Sclerosing poliodystrophy progressive SupranuclearPalsy Dystrophy Syndrome Refsum Disease infantile.
http://fayettemc-dl.slis.ua.edu/cgi/clickthrough.cgi/CT=http_3a_2f_2fwww_2eninds
National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health
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62. Harding: J Neurol Neurosurg Psychiatry, Volume 58(3).March 1995.pp 320-325.
Familial progressive poliodystrophy with cirrhosis of the liver. 3. Huttenlocher PR,Solitare GB, Adams G. infantile diffuse cerebral degeneration with hepatic
http://cloud.prohosting.com/lzambeni/prions/alpersteens.htm
Volume 58(3) March 1995 pp 320-325 Progressive Neuronal Degeneration of Childhood With Liver Disease (Alpers' Disease) Presenting in Young Adults [Papers]
Harding, B N; Alsanjari, N; Smith, S J M; Wiles, C M; Thrush, D; Miller, D H; Scaravilli, F; Harding, A E.
National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. Department of Neuropathology, Department of Clinical Neurophysiology, Department of Clinical Neurology, Derriford Hospital, Plymouth, UK.
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  • [Background] Keywords: progressive neuronal degeneration of childhood; liver disease; Alpers' disease Case reports Graphics Abstract Two unrelated and previously healthy girls, aged 17 and 18, presented with a subacute encephalopathy, visual and sensory symptoms and signs, and prominent seizures that were difficult to control. Brain MRI showed lesions (high signal on T2 weighted images) in the occipital lobes and thalamus; EEG showed slow wave activity with superimposed polyspikes. Inexorable downhill progression led to death in hepatic failure within eight months of onset. Histopathological findings in both patients ((a) chronic hepatitis with prominent bile duct proliferation, fatty change, and fibrosis; (b) in the brain a patchy destruction of the cerebral cortex, predominantly involving striate cortex) were characteristic of progressive neuronal degeneration of childhood with liver diseaseAlpers-Huttenlocher syndromea rare autosomal recessive disorder usually seen in infants and young children.

63. De Mitochondriale Ademhalingsketen Defecten
Klasse IV mutaties Ziekten van onbekende erfelijkheid. 1 Alpers Disease Progressiveinfantile poliodystrophy. 2 LIC Lethal infantile Cardiomyopathy.
http://www.homepages.hetnet.nl/~b1beukema/oxphos.html
De mitochondriële ademhalingsketen defecten de elektronentransportketen defecten de oxidatieve fosforylering Mitochondriële stoornissen betreffende de ademhalingsketen. (Respiratory Chain Defects ). Wat zijn mitochondriën Mitochondriën zijn kleine, roze kleurige, ovale of bolvormige organellen ( verzamelnaam voor kleine structuren in de cel ), ongeveer ter grootte van een kleine bacterie.( 1/1000 ste millimeter ). Onder een lichtmicroscoop zijn ze net zichtbaar. Ze zijn in aantallen van 1 tot honderden aanwezig in het cytoplasma ( protoplasma of vloeibare gedeelte van de cel) van alle menselijke cellen , behalve rode bloedlichaampjes.Grotere aantallen zijn er voornamelijk te vinden in de organen in het lichaam die veel energie nodig hebben , zoals het hart, het brein,de lever ,de skeletspieren, inwendige klieren en het beenmerg. Dit zijn dan ook de orgaansystemen die in het algemeen betrokken zijn bij de mitochondriële ziekten. Wat doen mitochondriën De mitochondriën zijn verantwoordelijk voor een groot aantal celstofwisselingfuncties, maar de belangrijkste celstofwisselingfunctie is de energie producerende functie.

64. Birth Disorder Information Directory - A
Alpers Disease (Alpers progressive Sclerosing poliodystrophy, Diffuse CerebralDegeneration in Infancy, Poliodystrophia Cerebri Progressiva/progressive
http://www.bdid.com/defectag.htm

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Ag-Am
Aganglionosis

65. Error Page
DISEASE Alpers progressive sclerosing poliodystrophy, Children withAlpers poliodystrophy suffer myoclonus epilepsy, related to
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=726

66. NEUROSURGERY://ON-CALL
List the major cerebral degenerative disorders of childhood including progressivesclerosing poliodystrophy; spongy degeneration; infantile neuraxonal dystrophy;
http://www.neurosurgery.org/resident/curriculum/neurology.html
NEUROLOGY UNIT OBJECTIVES
Demonstrate an understanding of the neurologic examination, diagnostic neurologic testing, neurologic diseases and their treatment. COMPETENCY-BASED KNOWLEDGE OBJECTIVES: Junior and Middle Levels:
  • Discuss electroencephalography. Recognize normal and abnormal EEG patterns. Identify specific epileptic conditions by EEG findings.
  • Describe the principles of sensory evoked potential testing (SEPs). Discuss how SEPs may be useful diagnostically.
  • List the indications for using intraoperative SEP monitoring and describe in detail how the procedure may be performed.
  • Describe the principles of visual evoked potential testing (VEPs). Discuss how VEPs may be useful diagnostically.
  • Describe the principles of motor evoked potential testing (MEPs). Discuss how MEPs may be useful diagnostically.
  • List the indications for using intraoperative MEP monitoring and describe in detail how the procedure may be performed.
  • Discuss electromyographic (EMG) testing in detail. Describe how the testing is performed and review the diagnostic capabilities of EMG testing. Describe the EMG changes associated with neuromuscular pathology.
  • List the indications for using intraoperative EMG testing and describe in detail how the procedure may be performed.
  • 67. Vindex, De Vindplaats Van Het Nederlandse Web
    community outreach and support. http//genetics.about.com/science/genetics/library/blalp.htm.Alpers' progressive infantile Poliodystophy.
    http://www.vindex.nl/dir/Health/Conditions_and_Diseases/Neurological_Disorders/N

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    Conditions and Diseases Neurological Disorders Neurodegenerative Diseases Alpers'
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    Alpers' Disease
    Gevonden in rubriek: Health Conditions and Diseases Neurological Disorders Neurodegenerative Diseases ... Alpers' Omschrijving: Information sheet compiled by NINDS. http://www.ninds.nih.gov/health_and_medical/disorders/alpersdisease_doc.htm About com Gevonden in rubriek: Health Conditions and Diseases Neurological Disorders Neurodegenerative Diseases ... Alpers' Omschrijving: Symptoms, diagnosis, and treatment of Alpers' disease. Information on research, community outreach and support. http://genetics.about.com/science/genetics/library/blalp.htm Alpers' Progressive Infantile Poliodystophy Gevonden in rubriek: Health Conditions and Diseases Neurological Disorders Neurodegenerative Diseases ... Alpers' Omschrijving: Features listed for this disorder.

    68. THE LIGHTNING HYPERTEXT OF DISEASE.
    Diffuse poliodystrophy or gray matter gliosis. Clinically characterizedInfantile onset of progressive weakness and atrophy.
    http://www.pathinfo.com/cgi-bin/lh.cgi?tx=calle

    69. Untitled
    Familial infantile spongy glioneuronal dystrophy (progressive cerebralpoliodystrophy).” Neuropatol Pol. 1978 ; 16(4) 563-73.
    http://neurobio-www.uia.ac.be/neurobio/CJD/RefInternet/CJD_1976-1981.txt
    J Neurol. 1981;
    Acta Pathol Jpn. 1981 ;
    J Neurol Neurosurg Psychiatry. 1981 ;
    Neurology. 1981 ;
    Acta Virol. 1981 ;
    Lancet. 1981 24;
    Lancet. 1981 21;
    Lancet. 1981 14;
    Ann Neurol. 1981 ;
    Neuropathol Appl Neurobiol. 1981 ;
    Sem Hop. 1981 8 15; Infect Control. 1981 ; Manuelidis, L. and E. E. Manuelidis (1981). “Search for specific DNAs in Creutzfeldt-Jakob infectious brain fractions using "nick translation".” Virology. 1981 ; Neurology. 1981 ; Arch Neurol. 1981 Jul; Surv Ophthalmol. 1981 ; Arch Neurol. 1981 ; Rev Neurol Paris. 1981; EEG EMG Z Elektroenzephalogr Verwandte Geb. 1981 ; (4): 205-11 ISSN: 0012-7590. Brain. 1981 ; Proc Soc Exp Biol Med. 1981 ; J Neurol Sci. 1981 ; Br Med J Clin Res Ed. 1981 14; Postepy Hig Med Dosw. 1981 ; Ital J Neurol Sci. 1981 ; Ital J Neurol Sci. 1981 ; Rinsho Shinkeigaku. 1981 ; Rinsho Shinkeigaku. 1981 ; Nervenarzt. 1981 ; Arch Psychiatr Nervenkr. 1981; Acta Pathol Jpn. 1981 ; Acta Pathol Jpn. 1981 ; Acta Pathol Jpn. 1981 ; J Neurol Sci. 1981 ; Virologie. 1981 Jul ; Virologie. 1981 Jul ; Ned Tijdschr Geneeskd. 1981 25;

    70. References
    Ayyar R (1977) Familial poliodystrophy, mitochondrial myopathy in autosomal dominantprogressive external ophthalmoplegia S (1992) Fatal infantile liver failure
    http://herkules.oulu.fi/isbn9514255674/html/b1247.html
    Phylogenetic analysis of mitochondrial DNA: Detection of mutations in patients with occipital stroke Prev
    References
    Avise JC (1986) Mitochondrial DNA and the evolutionary genetics of higher animals. Philosophical Transactions of the Royal Society of London - Series B: Biological Sciences. 312: 325-342. Bardosi A, Creutzfeldt W, DiMauro S, Felgenhauer K, Friede RL, Goebel HH, Kohlschutter A, Mayer G, Rahlf G, Servidei S, et al (1987) Myo-, neuro-, gastrointestinal encephalopathy (MNGIE syndrome) due to partial deficiency of cytochrome-c-oxidase. A new mitochondrial multisystem disorder. Acta Neuropathol 74: 248-258. Bille B (1997) A 40-year follow-up of school children with migraine. Cephalgia 17: 488-491. Bogenhagen DF (1999) Repair of mtDNA in vertebrates. Am J Hum Genet 64: 1276-1281. Chomyn A (1998) The myoclonic epilepsy and ragged-red fiber mutation provides new insights into human mitochondrial function and genetics. Am J Hum Genet 62: 745-751. Clayton DA (1982) Replication of animal mitochondrial DNA. Cell 28: 693-705. Clayton DA (1984) Transcription of the maintain mitochondrial genome. Annu Rev Biochem 53: 573-594.

    71. P
    Failure; @ Primary progressive Aphasia; @ Progeria; @ progressive InfantilePoliodystrophy; @ progressive Pallid Degeneration Syndrome;
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    72. Alpers Disease
    An article about Alpers' disease, including the alternate names, symptoms and statistics.Category Health Conditions and Diseases Alpers......Alpers Disease Alpers Disease, formally known as Alpers progressive InfantilePoliodystrophy, is a progressive neurodegenerative disease.
    http://www.cs.jmu.edu/common/projects/mtDNA/alpersdisease.html
    Alpers Disease Alpers Disease, formally known as Alpers Progressive Infantile Poliodystrophy, is a progressive neurodegenerative disease. It is also known as Christensen-Krabbe Disease. The symptoms initially manifest via convulsions. Over time, the symptoms grow to include dementia, spasticity, and monoclonus. The final symptom the patient usually experiences is status epilepticus (Ford, 1951). Following the initial convulsions, neuronal degeneration begins to occur. The degeneration results in the formation of spongiform tissue. Alpers is also linked to massive liver failures (Catherine, 1999). Back

    73. Privattandvård I Örebro, Tandläkaren Michel Deaibes, Sjukdomslista På Engels
    Primary Immune Disorders. Primary Ovarian Failure. Progeria. progressive InfantilePoliodystrophy. Prostate Cancer. Prostatic Hypertrophy. Prostatitis. Proteinuria.
    http://www26.brinkster.com/privtand/sjuk.htm
    A Aarskog Syndrome Aase Syndrome Abetalipoproteinemia Ablepharon-Macrostomia Syndrome Achilles Tendonitis Achondroplasia Acoustic Neuroma Acromegaly Activated Protein C Resistance Acute Idiopathic Polyneuritis ADD and ADHD Addiction and Recovery Addison's Disease Adiposis Dolorosa Adjustment Disorders Adrenoleukodystrophy Agnosia Agoraphobia Aicardi Syndrome AIDS Alagille Syndrome Albinism Alcoholism Alexander Disease Alkaptonuria Allergies Alopecia Alpers' Disease Alpha1 Antitrypsin Deficiency Alport Syndrome Alstrom Syndrome Alternating Hemiplegia Altophobia Alzheimer's Amblyopia Amputee Amyloidosis Amyoplasia Congenita Amyotrophic Lateral Sclerosis Anal Fissures Anemia Anencephaly Aneurysm Angina Pectoris Anophthalmos Anorexia Anosmia Anterior Knee Pain Syndrome Antiphospholipid Syndrome Anxiety Aortic Valve Disease Apert Syndrome Aphasia Aplastic Anemia Apnea, Sleep Appendicitis Arrhythmia Arteriohepatic Dysplasia Arthritis Arthrogryposis Asbestosis Asperger's Syndrome Aspergillosis Asthma Atherosclerosis Athlete's Foot Atrial Fibrillation Attachment Disorder Attention Deficit Disorder Autism Auto Immune Disorders Aviophobia Aviatophobia B Bacillary Angiomatosis Back Disorders Bad Breath Balanitis Baldness Barth Syndrome Bassen Kornzweig Syndrome Batten Disease Beckwith-Wiedemann Syndrome Behcet's Syndrome Bell's Palsy Benign Breast Lumps Benign Prostatic Hyperplasia Berger's Disease Beriberi Beryllium Disease Besnier Boeck Disease Betalipoprotein Deficiency Disease

    74. NINDS - Health And Medical
    E, Early infantile Epileptic Encephalopathy Empty Sella Syndrome Encephalitis andMeningitis Encephaloceles Encephalotrigeminal Angiomatosis Epilepsy Erb's
    http://nindsupdate.ninds.nih.gov/health_and_medical/research.htm
    National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health
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    75. Genetic Disorder And Birth Defects-aarskog,aase-smith,
    Alpers Syndrome ..progressive InfantilePoliodystrophy). Alpers Syndrome at UMDF http//www.umdf.org
    http://aspin.asu.edu/geneinfo/a.htm

    76. Medical Transcription Resources And Medical Books At BooksForMTs - Medical Terms
    Prognathism Prognosis Programmed cell death progressive progressive infantilepoliodystrophy progressive multifocal leukoencephalopathy Prokaryote
    http://www.booksformts.com/medterms_pTEXT.html
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    77. Searchalot Directory For Alpers'
    Information on research, community outreach and support. Alpers' ProgressiveInfantile Poliodystophy Features listed for this disorder.
    http://www.searchalot.com/Top/Health/ConditionsandDiseases/NeurologicalDisorders
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    78. Related Diseases

    http://www.csc.liv.ac.uk/~u1ej/others.htm
    Related Diseases - Provided by Neuroguide.com
    Acid Maltase Deficiency Acquired Epileptiform Aphasia Acute Disseminated Encephalomyelitis ADHD Adrenoleukodystrophy Affective Disorders (Depression, Manic-Depression) Agenesis of the Corpus Callosum Agnosia Aicardi Syndrome AIDS - Neurological Manifestations Alcoholism Alexander Disease Alpers' Disease Alternating Hemiplegia of Childhood Alzheimer's Disease Amyotrophic Lateral Sclerosis (ALS) Anencephaly Aneurysm Angelman Syndrome Angiomatosis Anosmia Anoxia or Hypoxia Anxiety and Panic Disorders Aphasia Apraxia Arachnoid Cysts Arachnoiditis Arnold-Chiari Malformation Arteriovenous Malformation Asperger Syndrome Ataxia Atrophy Attention Deficit Disorder Autism Autonomic Disorders Back Pain and Disk Disease Batten Disease Behcet's Disease Bell's Palsy Benign Essential Blepharospasm Benign Focal Amyotrophy Benign Intracranial Hypertension Binswanger's Disease Blepharospasm Blindness Botulism Brachial Plexus Birth Injuries Brachial Plexus Injuries Brain Aneurysm Brain Injury Brain Tumors Brown-Sequard Syndrome Canavan Disease Carpal Tunnel Syndrome Causalgia Central Pain Syndrome Cephalic Disorders Cerebral Aneurysm Cerebral Arteriosclerosis Cerebral Atrophy Cerebral Gigantism Cerebral Palsy Cervical Dystonia Charcot-Marie-Tooth Disease Chiari Malformations Chorea Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Chronic Pain Chronic Regional Pain Syndrome Chronic Fatigue Syndrome Clinical Trials Lists Coffin Lowry Syndrome Coma and Vegetative State Congenital Facial Diplegia Corticobasal Degeneration

    79. WebGuest - Open Directory Health Conditions And Diseases
    Alpers' Disease Information sheet compiled by NINDS. Alpers' ProgressiveInfantile Poliodystophy - Features listed for this disorder.
    http://directory.webguest.com/index.cgi/Health/Conditions_and_Diseases/Neurologi

    80. P
    The summary for this Chinese (Simplified) page contains characters that cannot be correctly displayed in this language/character set.
    http://www.wjgnet.com/wcjd/WCJDmc/1997/12/17.htm
    P.O.Box 2345, Beijing 100023,China Xin Xiaohuabingxue Zazhi
    Fax:+86-10-85381893 Tel: +86-10-85381892 ISSN
    Email: wcjd@public.bta.net.cn www.wjgnet.com
    HBV PBMC
    Mitochondrial function changes of peripheral blood mononuclear cells infected with HBV
    ZHANG Shu-Lin, WU Ning-Qiang, LIANG Xue-Song and DI Peng-Chao Hepatitis Lab, First Clinical College, Xi an Medical University, Xi an 710061, Shaanxi Province, China Subject headings hepatitis B/virology; hepatitis B virus/pathogenity; mitochondrial, liver/pathology; DNA,viral/analysis; polymerase chain reaction
    Abstract AIM
    To investigate the changes of mitochondrial function of peripheral blood mononuclear cells (PBMC) infected with HBV. METHODS Polymerase chain reaction was used to detect HBV DNA in PBMC and the MTT chromatometry was used to determine the mitochondrial function. HBV serum markers, IgM anti-HAV, anti-HCV were tested with radioimmunoassay. RESULTS Of 58 patients with chronic HBV infection, 31 (53.5%)

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