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Kearns-Sayre Syndrome MeSH Descriptor Data 2022


MeSH Heading
Kearns-Sayre Syndrome
Tree Number(s)
C05.651.460.700.500
C10.292.562.750.250.500
C10.597.622.447.511.500
C10.668.491.500.700.500
C11.590.472.250.500
C11.768.585.658.500.627
C14.280.238.510
C18.452.660.560.700.500
C23.550.291.500.688.500
C23.888.592.636.447.511.500
Unique ID
D007625
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D007625
Scope Note
A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)
Entry Term(s)
CPEO with Myopathy
CPEO with Ragged Red Fibers
Chronic Progressive External Ophthalmoplegia with Myopathy
Cpeo With Ragged-Red Fibers
Kearn-Sayre Mitochondrial Cytopathy
Kearns Sayre Syndrome
Kearns Syndrome
Kearns' Syndrome
Kearns-Sayre Mitochondrial Cytopathy
Kearns-Sayre-Shy-Daroff Syndrome
Oculocraniosomatic Syndrome
Ophthalmoplegia Plus Syndrome
Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy
Ophthalmoplegia, Progressive External, With Ragged-Red Fibers
Ophthalmoplegia-Plus Syndrome
Previous Indexing
Heart Block (1966-1982)
Ophthalmoplegia (1966-1982)
Retinitis Pigmentosa (1966-1982)
Public MeSH Note
2010; see KEARNS-SAYER SYNDROME 2000-2009, see KEARNS SYNDROME 1991-2004, see OPHTHALMOPLEGIA 1983-1990
History Note
2010 (1983)
Date Established
1991/01/01
Date of Entry
1982/04/26
Revision Date
2021/06/01
Kearns-Sayre Syndrome Preferred
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