Extractions: (advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Ophthalmology Neurologic Disorders Last Updated: February 2, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: abiotrophic ophthalmoplegia, CPEO with ragged red fibers, oculocraniosomatic neuromuscular disease, ocular myopathy, Olson disease, Kearns-Sayre-Daroff syndrome, Kearns-Sayre syndrome, progressive external ophthalmoplegia plus AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Extractions: Kearns-Sayre Syndrome,KSS,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 Kearns-Sayre Syndrome is a rare neuromuscular disorder characterized by three primary findings: progressive paralysis of certain eye muscles (chronic progressive external ophthalmoplegia [CPEO]); abnormal accumulation of colored (pigmented) material on the nerve-rich membrane lining the eyes (atypical retinitis pigmentosa), leading to chronic inflammation, progressive degeneration, and wearing away of certain eye structures (pigmentary degeneration of the retina); and heart disease (cardiomyopathy) such as heart block.
NeuroGate.com Search results for "chronic progressive external ophthalmoplegia" NO MATCHES FOUNDPlease select a different Search AltaVista for 'chronic progressive external ophthalmoplegia' http://www.neurogate.com/neuro/result.php3?search=Chronic Progressive External O
Extractions: (advertisement) Synonyms, Key Words, and Related Terms: abiotrophic ophthalmoplegia, CPEO with ragged red fibers, oculocraniosomatic neuromuscular disease, ocular myopathy, Olson disease, Kearns-Sayre-Daroff syndrome, Kearns-Sayre syndrome, progressive external ophthalmoplegia plus Background: Chronic progressive external ophthalmoplegia (CPEO) is a disorder characterized by slowly progressive paralysis of the extraocular muscles. Patients usually experience bilateral, symmetrical, progressive ptosis, followed by ophthalmoparesis months to years later. Ciliary and iris muscles are not involved. CPEO is the most frequent manifestation of mitochondrial myopathies. CPEO in association with mutations in mitochondrial DNA (mtDNA) may occur in the absence of any other clinical sign, but usually it is associated with skeletal muscle weakness. Kearns-Sayre syndrome (KSS) is a related mitochondrial myopathy that demonstrates the following: CPEO, onset before age 20, and pigmentary retinopathy. KSS also has at least one of the following: cardiac conduction defects, cerebrospinal fluid (CSF), protein of greater than 100 mg/dL, and a cerebellar syndrome. Other abnormalities in KSS can include mental retardation, Babinski sign, hearing loss, seizures, short stature, delayed puberty, and various endocrine disorders. CPEO also can be a sign in the following disorders: oculopharyngeal dystrophy, myasthenia gravis, and Graves disease.
Extractions: These patients frequently present with ptosis, ophthalmoplegia and mitochondrial myopathy. Severe cases have onset before 20 years of age and also manifest retinitis pigmentosa and a least one of the following: cardiac conduction defects, cerebellar ataxia, or elevated CSF protein above 100 mg/dl. Approximately 83% of KSS and 47% of CPEO patients carry mitochondrial DNA rearrangements detectable by southern analysis. A great majority of these rearrangements are new mutations. Therefore, the patients are heteroplasmic for normal and rearranged mitochondrial DNA molecules. The remainder of cases result from a variety of point mutations. Since mutant mitochondrial DNA molecules may be undetectable in blood cells due to replicative segregation, analysis of muscle biopsy may be necessary if the blood sample is negative for rearrangements or mutations. Blood 5-10 ml whole blood in an EDTA ( purple top ) or ACD ( yellow top ) tube.
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Extractions: Clin Cardiol. 1997 Mar; 20(3): 239-243. 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.
BioMed Central PDF Chronic Progressive External Ophthalmoplegia home journals AZ journals by subject advanced search authors reviewers libraries jobs about my BioMed Central. To obtain access to Current Neurology and Neuroscience Reports through your institution use the options below. http://www.biomedcentral.com/content/pdf/cr-nr2521.pdf
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?db=PubMed&cmd=Retrieve&dopt=Citati
Entrez PubMed Click here to read chronic progressive external ophthalmoplegia is associatedwith a novel mutation in the mitochondrial tRNA(Asn) gene. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Retrieve&dopt=Citati
CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA (CPEO) A CASE REPORT 96th DOG Annual Meeting, 1998. P521. chronic progressive external ophthalmoplegia (CPEO) A CASE REPORT. K. Meyer1, Ch. HaßTeller1, Ch. Matzen1, S. Günzel2, R. Schober2, P. Wiedemann1 The diagnosis of chronic progressive external ophthalmoplegia (CPEO) was established pathologically http://www.dog.org/1998/e-abstract98/521.html
Extractions: 96th DOG Annual Meeting, 1998 CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA (CPEO) A CASE REPORT K. Meyer , Ch. Haß-Teller , Ch. Matzen , S. Günzel , R. Schober P. Wiedemann Mitochondrial myopathies are a group of disorders that can cause a variety of multisystem signs and symptoms, like hereditary oculopharyngeal dystrophy, cerebellar dysfunction, epilepsy or heart block. The most important ophthalmologic features of these disorders include ptosis, progressive external ophthalmoplegia and pigmentary retinal degeneration. Klinik und Poliklinik für Augenheilkunde der Universität Leipzig, Liebigstr. 10-14, D-04103 Leipzig Abteilung für Neuropatholoqie der Universität Leipzig, Liebigstr. 26, D-04103 Leipzig Back
Extractions: Chronic progressive external ophthalmoplegia (CPEO) is a descriptive term for a heterogenous group of disorders characterized by chronic, progressive, bilateral, and usually symmetric ocular motility deficit and ptosis. Significant pain, proptosis, or pupil involvement are not features of CPEO and should prompt evaluation for alternative etiologies. Mitochondrial DNA mutations are increasingly being recognized as the etiology for CPEO syndromes. Clinicians should recognize the specific syndromes associated with CPEO, characterized by variable systemic, neurologic, or other findings. Treatment is limited, but newer therapies are being investigated.
Extractions: Department of Radiology, the University of New Mexico, Albuquerque, USA. PURPOSE: Our goal was to determine whether the extraocular muscles in patients with chronic progressive external ophthalmoplegia (CPEO) could be distinguished from those of age-matched control subjects by MR imaging. METHODS: Nine patients with CPEO and eight age-matched healthy control subjects were studied. The extraocular muscles of eight of the patients (16 eyes) and all the control subjects (16 eyes) were measured digitally. Images consisted of 1.5-mm contiguous sections acquired using a volume (three-dimensional) gradient-echo acquisition. In all, measurements were
Extractions: WWW Medical.WebEnds.com 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)