Extractions: Outline 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.
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
Extractions: 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.
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
Extractions: HOME Aganglionosis Aganthia Agammaglobulinemia Agenesis of the Cerebellar Vermis Agenesis of the Corpus Callosum Aglossia Adactylia (Oromandibular Limb Hypoplasia) Agnathia Agyria-Pachygyria Aicardi Syndrome Aicardi-Goutieres Syndrome (Encephalopathy-Basal Ganglia Calcification) Aksu Stockhausen Syndrome (Branchial Arch Defects) Al-Awadi Farag Teebi Syndrome (Primary Hypogonadism with Partial Alopecia) Al awadi farag teebi syndrome HYPOGONADISM, PRIMARY, AND PARTIAL ALOPECIA
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
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
Extractions: 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.
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
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
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
Extractions: Phylogenetic analysis of mitochondrial DNA: Detection of mutations in patients with occipital stroke Prev 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.
Alpers Disease An article about Alpers' disease, including the alternate names, symptoms and statistics.Category Health Conditions and Diseases AlpersAlpers Disease Alpers Disease, formally known as Alpers progressive InfantilePoliodystrophy, is a progressive neurodegenerative disease. http://www.cs.jmu.edu/common/projects/mtDNA/alpersdisease.html
Extractions: 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
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
Extractions: With your word-processing program or a text editor open, use your mouse to select all the words below in one group. Then, while the words are still selected, copy the words by keying <CTRL>+<C>. This copies the selected text to your computer's "clipboard." Go to a new page in your word-processing program or text editor and key <CTRL><V>. This "pastes" the words onto the page from your computer's "clipboard." Now run your spell-checking feature as you normally would and just add any words your spell-checker program does not recognize. The medical words listed below are separated by a "pipe" character. Please use the above list at your own risk. While we make every effort to ensure accuracy, we cannot be held responsible for errors in spelling or capitalization, or any circumstance that may result because of said error. In using the above list you also agree to never hold ANSO Consulting Inc. or BooksForMTs.com responsible for any errors should they exist. Book Lists Book Lists Menu Abbreviations Administration Atlases ... Writing Style
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
Extractions: Home Search News Email Greetings Weather ... Global All the Internet About AltaVista AOL Search Ask Jeeves BBC Search BBC News Business Dictionary Discovery Health Dogpile CheckDomain CNN Corbis eBay Education World Employment Encyclopedia Encarta Excite Fast Search FindLaw FirstGov Google Google Groups Infomine iWon Librarians Index Looksmart Lycos Metacrawler Microsoft Northern Light Open Directory SearchEdu SearchGov Shareware Teoma Thesaurus Thunderstone WayBackMachine Webshots WiseNut Yahoo! Yahoo! Auctions Yahoo! News Yahooligans Zeal Sponsored Links Top Health Conditions and Diseases Neurological Disorders ... Neurodegenerative Diseases : Alpers' Related Web Sites Alpers' Disease - Information sheet compiled by NINDS.
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
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
Extractions: 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%)
