A to Z medical terms definitions (medical terminology dictionary )
|
A |
B |
C |
D |
E |
F |
G |
H |
I |
J |
K |
L |
M |
N |
O |
P |
Q | |
R |
S |
T |
U |
V |
W |
X |
Y |
Z |
Definition of Kearns-Sayre syndrome
Kearns-Sayre syndrome definition - medical term
A neuromuscular disorder characterized by three
primary findings:
Progressive paralysis of certain eye muscles (chronic progressive external
ophthalmoplegia, or CPEO);
Abnormal accumulation of colored (pigmented) material on the retina (atypical
retinitis pigmentosa), leading to chronic inflammation and progressive degeneration
of the retina; and
Heart disease (cardiomyopathy) such as cardiac conduction defects and heart
block.
Other findings in the syndrome may include muscle weakness, short stature, hearing
loss, and the loss of ability to coordinate voluntary movements (ataxia) due to
problems in the part of the brain called the cerebellum.
Kearns-Sayre syndrome is one of the mitochondrial encephalomyopathies. These
disorders are due to defects in the DNA of the mitochondria, the cell structures
that produce energy. These defects cause the brain and muscles to function abnormally
(encephalomyopathy). In about 80% of cases of Kearns-Sayre syndrome, tests reveal
deletions in mitochondrial DNA (mtDNA).
There are many other names for the Kearns-Sayre syndrome including: CPEO with
myopathy; CPEO with ragged-red fibers; KSS; Mitochondrial cytopathy, Kearns-Sayre
type; Oculocraniosomatic syndrome; Ophthalmoplegia-plus syndrome; Ophthalmoplegia
with myopathy; and Ophthalmoplegia with ragged-red fibers.
medical
dictionary, online medical dictionary, medical terminology
dictionary, free online medical dictionary, medical
dictionary, online medical dictionary, medical terminology
dictionary, free online medical dictionary, dictionary
medical, dictionary medical online, dictionary medical
terminology, dictionary free medical online, dictionary free
medical, dictionary medical terms, dictionary drug medical
medicine, dictionary health illustrated medical nursing
profession stedmans, dictionary English medical, dictionary
medical online terminology, dictionary encyclopedia medical
online, condition dictionary medical, dictionary medical,
dictionary drug medical, medical dictionary software, cyclopedic
dictionary index medical tabers thumb,
medical dictionary, online medical dictionary, medical
terminology dictionary
