21. Arch Ophthalmol -- Abstract: Orbicularis Oculi Muscle In Chronic Progressive Ext Orbicularis oculi muscle in chronic progressive external ophthalmoplegia. J.Eshaghian, RL Anderson, TA Weingeist, MN Hart and PA Cancilla http://archopht.ama-assn.org/cgi/content/abstract/98/6/1070

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 98 No. 6, June 1980 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 Eshaghian J Cancilla PA Contact me when this article is cited Orbicularis oculi muscle in chronic progressive external ophthalmoplegia J. Eshaghian, R. L. Anderson, T. A. Weingeist, M. N. Hart and P. A. Cancilla Orbicularis oculi muscle biopsies were performed in 38 patients (ten with chronic progressive external ophthalmoplegia and 28 controls) to determine whether ragged red fibers were present and, if so, whether they were specific to progressive external ophthalmoplegia. To our knowledge, the orbicularis muscle has not been previously studied in this regard. Ragged

22. Arch Ophthalmol -- Abstract: Ultrastructural Analysis Of Extraocular Muscle In C For this reason, muscle samples obtained from surgery in a sporadic case ofchronic progressive external ophthalmoplegia (CPEO) were used for http://archopht.ama-assn.org/cgi/content/abstract/118/10/1441

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 118 No. 10, October 2000 Featured Link E-mail Alerts Clinicopathologic Report Article Options Full text PDF 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 Carta A Zeviani M Articles that cite this article ISI Web of Science (4) ... Contact me when this article is cited Topic Collections Ophthalmology, Other Topic Collection Alerts Ultrastructural Analysis of Extraocular Muscle in Chronic Progressive External Ophthalmoplegia Arturo Carta, MD Tiziana D'Adda, BSc Franco Carrara, BA Massimo Zeviani, MD, PhD Arch Ophthalmol. Extraocular muscles are primarily involved in many mitochondrial diseases, but no reports exist regarding the morphological appearance of the muscles in cases of long-standing ocular myopathies.

23. Kearns Sayre Syndrome chronic progressive external ophthalmoplegia and Myopathy; CPEO with Myopathy;CPEO with RaggedRed Fibers; KSS; Mitochondrial Cytopathy, Kearn-Sayre Type http://my.webmd.com/hw/raising_a_family/nord367.asp

var guid_source = ""; var guid_source_id = ""; //unused var encodedurl = ""; WebMD Today Home WebMD News Center XML News via RSS Member Services WebMD University My WebMD Find a Physician Medical Info Check Symptoms Medical Library Health Tools Clinical Trials ... Women, Men, Lifestyle Who We Are About WebMD Site Map Health Topics Symptoms ... For a Complete Report Kearns Sayre Syndrome Important It is possible that the main title of the report Kearns Sayre Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Chronic Progressive External Ophthalmoplegia and Myopathy CPEO with Myopathy CPEO with Ragged-Red Fibers KSS Mitochondrial Cytopathy, Kearn-Sayre Type Oculocraniosomatic Syndrome (obsolete) Ophthalmoplegia, Pigmentary Degeneration of the Retina and Cadiomyopathy Kearns-Sayre Disease Chronic Progressive External Ophthalmoplegia with Ragged Red Fibers Ophthalmoplegia Plus Syndrome Disorder Subdivisions None General Discussion This disease entry was made possible due to the generosity of the Robert Lee and Clara Guthrie Patterson Trust, through grant funds provided for the National Organization for Rare Disorders' "Pediatric Rare Disease Database Project."

24. Entrez PubMed chronic progressive external ophthalmoplegia a correlative study of quantitativemolecular data and histochemical and biochemical profile. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9

25. Entrez PubMed chronic progressive external ophthalmoplegia a correlative study of mitochondrialDNA deletions and their phenotypic expression in muscle biopsies. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9

26. Chronic Progressive External Ophthalmoplegia Topic - Unified Search Environment chronic progressive external ophthalmoplegia Topic Tree Progressive ExternalOphthalmoplegia MSH/EN/D017246 ICD9CM/PT/378.72 MSH/PM/D017246 http://www.use.hcn.com.au/portals/shared/subject.`Chronic progressive external o

Chronic progressive external ophthalmoplegia Topic Tree Definition: A mitochondrial myopathy characterized by slowly progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. Ragged-red fibers and atrophy are found on muscle biopsy. Familial and sporadic forms may occur. Disease onset is usually in the first or second decade of life, and the illness slowly progresses until usually all ocular motility is lost. (From Adams et al., Principles of Neurology, 6th ed, p1422) Synonyms and Source Vocabularies: Chronic progressive external ophthalmoplegia Progressive External Ophthalmoplegia CPEO Graefe Disease Ocular Muscular Dystrophy Mitochondrial Ocular Myopathy Ocular Myopathy of Von Graefe-Fuchs Progressive External Ophthalmoplegias Ocular Motility Disorders Duane Retraction Syndrome Nystagmus, Pathologic Oculomotor Nerve Paralysis ... Tolosa-Hunt Syndrome Chronic progressive external ophthalmoplegia Kearns-Sayre syndrome Miller Fisher Syndrome Ophthalmoplegia Diplopia ... Supranuclear Palsy, Progressive Chronic progressive external ophthalmoplegia Kearns-Sayre syndrome Mitochondrial Myopathies Mitochondrial Encephalomyopathies Chronic progressive external ophthalmoplegia Kearns-Sayre syndrome

27. Chronic Asymmetric Progressive External Ophthalmoplegia With Right Facial Weakne Chronic asymmetric progressive external ophthalmoplegia with right facial weaknessa unique presentation chronic progressive external ophthalmoplegia. http://jnnp.bmjjournals.com/cgi/content/full/73/1/95

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Extract Full Text (PDF) Submit a response ... 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 Sharma, N K Articles by Kattah, J C Related Collections Genetics Neuromuscular disease Journal of Neurology Neurosurgery and Psychiatry Journal of Neurology Neurosurgery and Psychiatry LETTER Chronic asymmetric progressive external ophthalmoplegia with right facial weakness: a unique presentation of mitochondrial myopathy N K Sharma M Gujrati J Kumar and J C Kattah Department of Neurology, University of Illinois College of Medicine at Peoria, One Illini Drive, Box 1649, Peoria, Illinois 61656-1649, USA Correspondence to: Dr J C Kattah;

28. Two Families With Autosomal Dominant Progressive External Ophthalmoplegia -- Kie the late 1980s.1 Apart from chronic progressive external ophthalmoplegia, Mutation of POLG is associated with progressive external ophthalmoplegia http://jnnp.bmjjournals.com/cgi/content/full/75/8/1125

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Abstract Full Text (PDF) Submit a response ... Citation Map 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 Kiechl, S Articles by Willeit, J Related Collections Other Neurology Journal of Neurology Neurosurgery and Psychiatry BMJ Publishing Group Ltd PAPER Two families with autosomal dominant progressive external ophthalmoplegia S Kiechl P Luoma U Kiechl-Kohlendorfer B Wallacher-Scholz R Stucka C Thaler J Wanschitz A Suomalainen M Jaksch and J Willeit Department of Neurology, University Hospital Innsbruck, Innsbruck, Austria Metabolic Disease Center Munich-Schwabing and Departments of Clinical Chemistry, Academic Hospital Schwabing, Munich, Germany Department of Neurology and Programme of Neurosciences, Biomedicum Helsinki, University of Helsinki, Finland

29. A Case Of Chronic Progressive External Ophthalmoplesia With A Heteroplasmic A-to We report on a 35 yearold female with chronic progressive external ophthalmoplegia.She had bilateral blepharoptosis, total ophthalmoplegia and muscle http://www.shinkeiganka.com/search/e16_1_3.html

Leu(UUR) a case of chronic progressive external ophthalmoplesia with a heteroplasmic A-to-G transition at nt.3243 in the tRNA Leu(UUR) Yoshiko Aoki, Yasuhiro Nishida, Toyotaka Murata, Makiko Kimura, Masahiko Terada We report on a 35 year-old female with chronic progressive external ophthalmoplegia. She had bilateral blepharoptosis, total ophthalmoplegia and muscle weakness of the bilateral proximal groups of the upper extremities and facial muscles. She did not exhibit either endocrinological disorder or hearing loss nor was there any family history. Pathological findings of the limb skeletal muscle showed ragged red fibers and cytochrome c oxidase-negative fibers. Mitochondrial DNA analysis revealed a mutation at the heteroplasmic A-to-G transition at nt.3243 in the RNA (LeuUUR) . During clinical examinations, there were no signs of encephalopathy or stroke-like episodes normally seen in cases of MELAS. keyword : CPEO, mtDNA3243 tRNA (LeuUUR) mutation, MELAS

30. Accessing Article chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial myopathycharacterised by ophthalmoplegia and ptosis. http://www.nature.com/eye/journal/vaop/ncurrent/full/6701924a.html

NATURE.COM NEWS@NATURE.COM NATUREJOBS NATUREEVENTS ... Help E-MAIL: PASSWORD: Save password Forgotten password? Most users gain access to full text articles through a site license. This is available to institutional customers only. For further information visit the Librarian Gateway. Full-text articles are also available through a number of other options: I want to purchase this article Select this option to view this article immediately and access it for seven days. Price: US$30* In order to purchase this article you must be a registered user. Click here to register or log in above. I want to subscribe to Eye Select this option to purchase a personal subscription. I am already a personal subscriber to Eye Personal subscribers to Eye can view this article. To do this you need to associate your subscription with your registration via the My Account page. If you already have an active online subscription, log in via the login box in the top right-hand corner of this page. I am a member of The Royal College of Ophthalmologists Members of The Royal College of Ophthalmologists receive access to Eye as part of their membership.

31. Accessing Article We studied 25 patients with chronic progressive external ophthalmoplegia. In eachcase muscle biopsies were consistent with mitochondrial myopathy. http://www.nature.com/eye/journal/v19/n3/full/6701488a.html

NATURE.COM NEWS@NATURE.COM NATUREJOBS NATUREEVENTS ... Help E-MAIL: PASSWORD: Save password Forgotten password? Most users gain access to full text articles through a site license. This is available to institutional customers only. For further information visit the Librarian Gateway. Full-text articles are also available through a number of other options: I want to purchase this article Select this option to view this article immediately and access it for seven days. Price: US$30* In order to purchase this article you must be a registered user. Click here to register or log in above. I want to subscribe to Eye Select this option to purchase a personal subscription. I am already a personal subscriber to Eye Personal subscribers to Eye can view this article. To do this you need to associate your subscription with your registration via the My Account page. If you already have an active online subscription, log in via the login box in the top right-hand corner of this page. I am a member of The Royal College of Ophthalmologists Members of The Royal College of Ophthalmologists receive access to Eye as part of their membership.

32. Chronic Progressive External Ophthalmoplegia With Ragged Red ThirdAid.com the world s first dedicated online patient to patient medical helpcommunity. http://www.thirdaid.com/conditions/Chronic_Progressive_External_Ophthalmoplegia_

33. Mental Diseases (KearnsSayre Disease , chronic progressive external ophthalmoplegia and Myopathy ,Mitochondrial Cytopathy, Kearn-Sayre Type , CPEO with Ragged-Red Fibers http://www.thirdaid.com/index/qfm/fuseaction/registrationStep3/catalog_id/10521/

34. Ophthalmic Hyperguide. Oculoplastics: Ophthalmoplegia Plus Syndromes chronic progressive external ophthalmoplegia may also occur in isolation of anyother system involvement. To correctly diagnose the underlying disorder in a http://www.ophthalmic.hyperguides.com/tutorials/oculoplastics/ophthalmoplegia_pl

Search: HELP Tutorial Introduction Mitochondrial Genetics Mitochondrial CPEO Kearns-Sayre Syndrome MELAS and MNGIE Diagnostic Considerations Bibliography Slides Ophthalmoplegia Plus Syndromes Janet Rucker, MD Introduction Neurologic, ophthalmologic, and systemic disorders with common chronic progressive external ophthalmoplegia (CPEO) have been identified. Chronic progressive external ophthalmoplegia may also occur in isolation of any other system involvement. To correctly diagnose the underlying disorder in a patient with CPEO, a baseline understanding of the common disorders and pathways to diagnosis is necessary. The term ophthalmoplegia plus was coined by David Drachman, MD, in 1968 and refers to disorders with abnormalities, often neurologic, in addition to CPEO. Knowledge of these disorders has increased significantly since then; however, overlap between clinical phenotypes and genetic defects for the most common causes of CPEO remains. Chronic progressive external ophthalmoplegia should be considered a sign of disease, rather than a disease. In addition to slowly progressive, bilateral, typically symmetric ocular immobility, bilateral ptosis is often present ( Slide 1 ). Mitochondrial myopathy is the most common cause of CPEO, but CPEO may be a component of other disorders that should be considered in the differential diagnosis (Table 1).

35. Ophthalmic Hyperguide. Section: Oculoplastics chronic progressive external ophthalmoplegia is a disorder resulting from a defectin the mitochondrial function in which the extraocular and periocular http://www.ophthalmic.hyperguides.com/tutorials/oculoplastics/ptosis/tutorial.as

Search: HELP Pretutorial Assessment Tutorial Introduction Aponeurotic Ptosis Neurogenic Ptosis Myogenic Ptosis Traumatic Ptosis Mechanical Ptosis Pseudoptosis Evaluation of Ptosis Photography Consultation References Slides Classification and Examination of Patients with Acquired Ptosis Mark R. Levine, MD Introduction SLIDE 1 Over the years, many classifications of ptosis have been developed. However, recently with refinements in the diagnosis of ptosis, along with improved anatomic evaluation and study of the pathophysiology of certain types of ptosis, a better understanding of ptosis has developed. Ptosis may be classified into congenital and acquired types. Congenital ptosis is a developmental dystrophy of the levator muscle of an unknown cause ( Slide 1 SLIDE 2 The condition is usually sporadic, but it may be hereditary. Congenital ptosis may be simple with the defect isolated to only the levator muscle or with superior rectus muscle weakness. Congenital ptosis also includes blepharophimosis syndrome, which is hereditary and which, in addition to ptosis, may include varying degrees of telecanthus, epicanthus inverses, phimosis, and ectropion of the lower eyelids. Congenital ptosis also includes the Marcus Gunn jaw winking syndrome, which is caused by abnormal levator innervation rather than a striated muscle fiber deficiency. Acquired ptosis is best classified by Beard ; however, other authors have added subclassifications, including aponeurotic, neurogenic, myogenic, mechanical, traumatic, and pseudoptosis.

36. British Library Direct: Order Details Order from the British Library chronic progressive external ophthalmoplegia,mitochondrial encephalomyopathies, and ocular motor disorders Japanese http://direct.bl.uk/research/50/33/RN140445083.html

This is an article from British Library Direct, a new service that allows you to search across 20,000 journals for free and order full text using your credit card. Buy this article Search British Library Direct Return to Google Article details Article title Chronic progressive external ophthalmoplegia, mitochondrial encephalomyopathies, and ocular motor disorders Author Kase, M. Journal title JAPANESE JOURNAL OF CLINICAL OPHTHALMOLOGY Bibliographic details 2003, VOL 57; PART 9, pages 1407-1419 Publisher IJAKU SHOIS LTD Country of publication Japan ISBN ISSN Language Original Pricing To buy the full text of this article you pay: service charge Abstract: By using this site you agree to our terms and conditions Privacy policy We welcome your comments

37. Cardiac Dysfunction In Patients With Chronic Progressive External Ophthalmoplegi Clin Cardiol. 1997 Mar; 20(3) 239243. Cardiac Dysfunction in Patients withchronic progressive external ophthalmoplegia http://www.clinicalcardiology.org/briefs/9703briefs/cc20-239.html

Search this site: Clin Cardiol. 1997 Mar; 20(3): 239-243. Cardiac Dysfunction in Patients with Chronic Progressive External Ophthalmoplegia M. Akaike, M.D., H. Kawai, M.D., K. Yokoi, M.D., M. Kunishige, M.D., H. Mine, M.D., Y. Nishida, M.D., S. Saito, M.D. The cardiac function in five patients with chronic progressive external ophthalmoplegia (CPEO) was evaluated. Carotid pulse recording and echocardiography showed a left ventricular systolic dysfunction in two patients. One of these patients died of congestive heart failure 2 months after the study. The Doppler pattern of left ventricular filling in the three remaining patients showed left ventricular diastolic dysfunction. Although the cardiac involvement of patients with CPEO is generally considered to be limited to the cardiac conduction system, left ventricular dysfunction should receive more attention in the management of patients with CPEO.

38. Muscular Dystrophy Campaign that most of these patients have either oculopharyngeal muscular dystrophy (OPMD)or mitochondrial chronic progressive external ophthalmoplegia (CPEO). http://www.muscular-dystrophy.org/information_resources/factsheets/medical_condi

About us News About your condition Research ... Community Accessibility Print Home Donate Sitemap Contact Ocular myopathies Written by Dr D.Hilton-Jones, Consultant Neurologist, Oxford for the Muscular Dystrophy Campaign. It has been recognised for many years that some patients with muscle disease have particular problems with the muscles around the eyes, although other parts of the body can also be involved. Whilst research is continuing, it appears that most of these patients have either oculopharyngeal muscular dystrophy (OPMD) or mitochondrial chronic progressive external ophthalmoplegia (CPEO). The main features of these disorders are discussed below. Some of the symptoms and signs are common to both disorders. You can download this factsheet as a Word document: Ocular Myopathies factsheet Contents: Oculopharyngeal muscular dystrophy (OPMD) Symptoms and signs Management How is OPMD inherited? ... Diagnosis Oculopharyngeal muscular dystrophy (OPMD) Muscular dystrophy is a term used to describe a number of conditions in which there is progressive muscle weakness, caused by the patient having a faulty gene. In OPMD the weakness mainly affects the ocular (eye) and pharyngeal (throat) muscles. To Top Symptoms and signs Although the abnormal gene is present from birth, patients do not usually develop symptoms until the fifth or sixth decade of life. The first sign of the disorder is usually

39. Chronic Progressive External Ophthalmoplegia (CPEO) 9453 Ptosis; chronic progressive external ophthalmoplegia (CPEO) 906-2 Ptosis;chronic progressive external ophthalmoplegia (CPEO) http://medstat.med.utah.edu/NOVEL/tmp/Wray/CPEO.php

Chronic progressive external ophthalmoplegia (CPEO) Clinical signs Both the cardiac and endocrine complications of Kearns-Sayre Syndrome may be life threatening. The associated abnormalities, such as hearing loss, impaired intellect, and spongeform encephalopathy are summarized in Table 9-12, p381, Leigh and Zee) Presenting symptom Droopy eyelids ... 926-2 Progressive External Ophthalmoplegia

40. 1 In a patient with chronic progressive external ophthalmoplegia and bilateralptosis obscuring the visual axis. Would you perform levator resection or brow http://www.mrcophth.com/vivaquestions/viva1/3.html

In a patient with chronic progressive external ophthalmoplegia and bilateral ptosis obscuring the visual axis. Would you perform levator resection or brow suspension and why? Answer In chronic progressive external ophthalmoplegia, the patient has poor Bell's phenomenon. In addition, most of this patients eventually develop orbicularis oculi weakness. If levator resection was performed, there may be corneal expsoure when orbicularis oculi weakness develops due to unopposed levator function. Brow suspension is preferable. Even then it is important to be conservative due to the risk of corneal exposure. What are the complications of ptosis surgery? Answer Complications of any operations can be classified into those that occur in all operations and those that are specific to a particular operation. Haemorrhage and infections are the non-specific complilcations of all operations. Complications specific to ptosis surgery are: Over or under-correction and eyelid contour abnormalities. Corneal expsoure causing expsoure keratopathy Conjunctival prolapse Contralateral ptosis Orbital haemorrhage Following a sucessful ptosis surgery. A patient complains of blurred vision but examination shows no evidence of corneal abnormalites. What may be responsible for this?

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