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         Chronic Progressive External Ophthalmoplegia:     more detail

81. Ophthalmoplegia
As with most chronic neurologic diseases, mortality increases with disability. Progressiveexternal ophthalmoplegia itself is not a lifethreatening condition.
http://www.healthatoz.com/healthatoz/Atoz/ency/ophthalmoplegia.html
Encyclopedia Index O Home Encyclopedia Encyclopedia Index O Ophthalmoplegia
Definition
Ophthalmoplegia is a paralysis or weakness of one or more of the muscles that control eye movement. The condition can be caused by any of several neurologic disorders. It may be myopathic, meaning that the muscles controlling eye movement are directly involved, or neurogenic, meaning that the nerve pathways controlling eye muscles are affected. Diseases associated with ophthalmoplegia are ocular myopathy, which affects muscles, and internuclear ophthalmoplegia, a disorder caused by multiple sclerosis , a disease which affects nerves. Description Because the eyes do not move together in ophthalmoplegia, patients may complain of double vision. Double vision is especially troublesome if the ophthalmoplegia comes on suddenly or affects each eye differently. Because ophthalmoplegia is caused by another, underlying disease, it is often associated with other neurologic symptoms, including limb weakness, lack of coordination, and numbness. Causes and symptoms Ocular myopathy is also known as mitochondrial encephalomyelopathy with ophthalmoplegia or progressive external ophthalmoplegia. Because it is so often associated with diseases affecting many levels of the neurologic system, it is often referred to as "ophthalmoplegia plus." The main feature is progressive limitation of eye movements, usually with drooping of the eyelids (

82. Biochem. Soc. Trans (2000) Volume 28, Part 2 - Organisms, Organs, Cells And Orga
Abbreviations used mt DNA, mitochondrial DNA; CPEO, chronic progressive externalophthalmoplegia; KSS, Kearns–Sayre syndrome; COX, cytochrome c oxidase; CS
http://bst.portlandpress.com/bst/028/0159/bst0280159.htm
Full Text Author Keywords Title
Biochem. Soc. Trans. (2000) (Printed in Great Britain)
Muscle fibres: applications for the study of the metabolic consequences of enzyme deficiencies in skeletal muscle
Abbreviations used: c oxidase; CS, citrate synthase. To whom correspondence should be addressed. Abstract Introduction The metabolic consequences of large rearrangements of mitochondrial DNA in skeletal muscle of patients with mitochondrial myopathies remain unclear [ ]. These problems may be attributed to various reasons. First, the heteroplasmic occurrence of the mtDNA mutation; second, the unpredictable mosaic distribution of mtDNA mutations in the affected tissue; third, unknown individual effects of various mutations; and fourth, difficulties in the quantitative determination of respiratory chain-enzyme activities and the degree of heteroplasmy of the mtDNA in skeletal muscle. In contrast, in cell cultures containing well-defined amounts of heteroplasmic mtDNA for different mutations, so-called threshold values have been defined beyond which each mutation had an effect on the activity of mtDNA-encoded enzymes. For various tRNA point mutations this threshold has been determined to be above 85% of mutated mtDNA [ In this report we studied the metabolic consequences of deficiencies of enzymes of the mitochondrial respiratory chain in skeletal muscle by applying different techniques: enzyme-activity measurements with improved methods and investigation of saponin-permeabilized muscle fibres with respirometric, fluorimetric and imaging techniques.

83. Ask The Experts
MITOCHONDRIAL MYOPHATHIES. CPEO (CPEO) (chronic progressive externalophthalmoplegia); KSS (KSS) * updated Nov/02 (KearnsSayre Syndrome);
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MUSCULAR DYSTROPHIES

MOTOR NEURON DISEASES

84. JKMS Vol 4, No. 2
Abstract. Fulltext. 1989 Jun;4(2)91-96. chronic progressive externalophthalmoplegia (CPEO) with 'ragged red fibers'a case report.
http://jkms.kams.or.kr/1989/abstract/91a.html
Abstract Full-text 1989 Jun;4(2):91-96
Chronic progressive external ophthalmoplegia (CPEO) with 'ragged red fibers'a case report.
Kim JS, Kim CJ, Chi JG, Myung HJ
Department of Neurology, Asan Medical Center, Seoul, Korea.
Chronic progressive external ophthalmoplegia (CPEO) is a rare clinical syndrome characterized by slowly progressive paralysis of extraocular muscles. We report a male patient who had a 20 year history of CPEO. Histological examination of left deltoid muscle showed characteristic ragged red fibers. Electron microscopy revealed a number of abnormal mitochondria which contain paracrystalline inclusion bodies.

85. Pubblicazioni Gerli
Morphometric and biochemical study of muscle mitochondria in adult chronicprogressive external ophthalmoplegia. J. Inher. Metab. Dis. 11 Suppl.
http://www.unisi.it/ricerca/dottorationweb/sc-morfol-linf/pubblicazionegerli.HTM
ALCUNE PUBBLICAZIONI DEL PROF.GERLI R. Gerli , G. Pompucci, C. Fruschelli. Enzime activities of the anaerobic glycolisis in the lymph vascular wall. 3th International Congress of Lymphology , Bruxelles (Belgium), September 3-5, 1970. R. Gerli , G. Pompucci, C. Fruschelli. Enzime activities of the pentose phosphate pathway in the lymph vascular wall. 3th International Congress of Lymphology , Bruxelles (Belgium), September 3-5, 1970. R. Baiocchi, R. Andreassi, E. Sbano, C. Benedetti, M. Fimiani, R. Gerli , C. Alessandrini. Neoplasia glomangiomiforme in corso di leucosi linfatica cronica. Ann.It.Derm.Clin.Sper R. Gerli , G. Sacchi, C. Alessandrini, L. Ibba, A.M. Pucci, C. Fruschelli. Ricerche istochimiche ed ultrastrutturali sull'innervazione della parete vascolare linfatica e sanguifera: I) L'innervazione adrenergica. Boll.Soc.Ital.Sper ., Vol.LV, n.22, 2281-2287, 1979. R. Gerli , G. Sacchi, C. Alessandrini, A.M. Pucci, L. Ibba, C. Fruschelli. Ricerche istochimiche ed ultrastrutturali sull'innerva- zione della parete vascolare linfatica e sanguifera: II) L'innervazione colinergica.

86. A3243G - Abstract - A Case Of Mitochondrial Encephalomyopathy Showing Ophthalmop
We report a 47year-old female patient showing clinical features of chronicprogressive external ophthalmoplegia (CPEO) without stroke-like episodes.
http://www.a3243g.com/abstract_pm_9248343.asp
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Publication: Rinsho Shinkeigaku 1997 Apr;37(4):326-30 [A case of mitochondrial encephalomyopathy showing ophthalmoplegia, diabetes mellitus and hearing loss associated with the A3243G mutation of mitochondrial DNA]

[Article in Japanese]
Hoshino S, Tamaoka A, Ohkoshi N, Shoji S, Goto Y. Department of Neurology, University of Tsukuba. Original Abstract Date Page Updated: 14 September 2002 Email this page to a friend. Feedback on this page. We subscribe to the HONcode principles. Verify here Terms Privacy Funding

87. ? ?
The summary for this Japanese page contains characters that cannot be correctly displayed in this language/character set.
http://brain.bri.niigata-u.ac.jp/~stsuji/resrc/disease/muscle/cpeo/cpeo.htm
1. Introduction
  • ƒ~ƒgƒRƒ“ƒhƒŠƒA”]‹ØÇ‚ÌŠT”O
    • ƒ~ƒgƒRƒ“ƒhƒŠƒAƒ~ƒIƒpƒ`[‚Ƃ͍œŠi‹Ø“à‚ɈُíŒ`‘Ô‚ðŽ¦‚·ƒ~ƒgƒRƒ“ƒhƒŠƒA‚ª‘½””F‚ß‚ç‚ê‚鎾Š³‚Å‚ ‚éDƒGƒlƒ‹ƒM[Žù—v‚ª‘å‚«‚¢‘ŸŠíi‹ØC’†•‚¨‚æ‚Ñ––½_ŒoŒnCSCtj‚𒆐S‚É‘½‘ŸŠí‚ª–«‚©‚is«‚ɐN‚³‚êCƒ~ƒIƒpƒ`[C’†•_ŒoÇó‚ð‚Í‚¶‚ß‚Æ‚µ‚āC‘½Ê‚ȗՏ°Çó‚ð’æ‚·‚邱‚Æ‚©‚çCƒ~ƒgƒRƒ“ƒhƒŠƒA”]‹ØÇ‚Æ‚¢‚¤–¼‚Å‘Ì‚³‚ê‚éD @
    • ƒ~ƒgƒRƒ“ƒhƒŠƒA ^Šj¶•¨
  • ƒ~ƒgƒRƒ“ƒhƒŠƒA”]‹ØÇ‚Ì•ª—ށ@
  • –«is«ŠOŠá‹Ø–ƒáƒ(chronic progressive external ophthalmoplegia) Kearns-SayreÇŒóŒQ‚́CŠOŠá‹Ø–ƒáƒ‚ɉÁ‚¦C–Ô–ŒF‘f•Ï¬CS“`“±ƒuƒƒbƒN‚Ì3Žå’¥‚ð’æ‚·‚é‚à‚̂ŁCCPEOŠÜ‚Ü‚ê‚éD
  • ragged-red fiber‚𔺂¤ƒ~ƒIƒNƒ[ƒkƒX‚Ä‚ñ‚©‚ñ (MERRFF; myoclonus epilepsy associated with ragged-red fibers)
  • ‘²’†—lÇó‚𔺂¤ƒ~ƒgƒRƒ“ƒhƒŠƒA•a (MELAS: mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episode)
  • Leber•a (Leber's hereditary optic neuropathy)
  • “dŽq“`’BŒnˆÙí •¡‡‘Ì I Œ‡‘¹ •¡‡‘Ì II Œ‡‘¹ •¡‡‘Ì III Œ‡‘¹ •¡‡‘ÌIVŒ‡‘¹
  • “œ”A•a/“ï’® 7. Pearson•a 8. ƒ~ƒgƒRƒ“ƒhƒŠƒADNAŒ‡–RÇ@
    • –«‚ÌŒo‰ß‚ð‚Æ‚éƒ~ƒIƒpƒ`[‚Ì‘¶ÝC’ág’·CÇ—á‚É‚æ‚Á‚ẮCŠOŠá‹Ø–ƒáƒ (CPEO)C
    • ˆâ“`Šw“I‚È–Ê‚©‚ç‚́CMERRF, MELAS, Leber•a‚ɂ‚¢‚Ä‚Í•êŒnˆâ“`‚ªl‚¦‚ç‚ê‚ê‚΋­‚­‹^‚¤DCPEO‚ɂ‚¢‚ẮCŒÇ”­—Ⴊ‘½‚¢D‚Ü‚ê‚ɁCíõF‘Ì—D«ˆâ“`«‚Ì‚à‚Ì‚à‚ ‚éiŠj‚̃Qƒmƒ€DNA (íõF‘Ì) ã‚̈â“`ŽqˆÙí‚ÌŒ‹‰Êƒ~ƒgƒRƒ“ƒhƒŠƒADNA‚É‘½dŒ‡Ž¸‚ª¶‚¶‚â‚·‚­‚È‚é‚ƍl‚¦‚ç‚ê‚éj @

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