Search on: CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA 
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Descriptor English:   Ophthalmoplegia, Chronic Progressive External 
Descriptor Spanish:   Oftalmoplejía Externa Progresiva Crónica 
Descriptor Portuguese:   Oftalmoplegia Externa Progressiva Crônica 
Synonyms English:   Chronic Progressive External Ophthalmoplegia
Graefe's Disease
Ocular Myopathy of Von Graefe-Fuchs
Ophthalmoplegia, Progressive External
Progressive External Ophthalmoplegia
Disease, Graefe
Dystrophy, Ocular Muscular
External Ophthalmoplegia, Progressive
Muscular Dystrophies, Ocular
Muscular Dystrophy, Ocular
Myopathy, Mitochondrial Ocular
Ocular Muscular Dystrophies
Ocular Myopathy of Von Graefe Fuchs
Ocular Myopathy, Mitochondrial
CPEO
Graefe Disease
Mitochondrial Ocular Myopathy
Ocular Muscular Dystrophy  
Tree Number:   C05.651.460.700
C10.292.562.750.250
C10.597.622.447.511
C10.668.491.500.700
C11.590.472.250
C18.452.660.560.700
C23.888.592.636.447.511
Definition English:   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) 
Indexing Annotation English:   chronic progressive external ophthalmoplegia with cardiomyopathy & retinitis pigmentosa = KEARNS-SAYRE SYNDROME
History Note English:   93 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications CN congenital
DI diagnosis DG diagnostic imaging
DH diet therapy DT drug therapy
EC economics EM embryology
EN enzymology EP epidemiology
EH ethnology ET etiology
GE genetics HI history
IM immunology ME metabolism
MI microbiology MO mortality
NU nursing PS parasitology
PA pathology PP physiopathology
PC prevention & control PX psychology
RT radiotherapy RH rehabilitation
SU surgery TH therapy
UR urine VE veterinary
VI virology  
Record Number:   30631 
Unique Identifier:   D017246 

Occurrence in VHL:
 

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