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Choroid plexus failure in the Kearns-Sayre syndrome
SpectorandJohansonCerebrospinalFluidResearch2010,7:14
/content/7/1/14
CEREBROSPINAL FLUID RESEARCH
COMMENTARY
OpenAccess
ChoroidplexusfailureintheKearns-Sayre
syndrome
ReynoldSpector1,ConradEJohanson2*
Abstract
TheKearns-Sayresyndromeisamitochondrialdisorder(generallyduetomitochondrialDNAdeletions)thatcauses
ophthalmoplegia,retinopathy,ataxiaandbrainabnormalitiessuchasleukoencephalopathy.Inthissyndrome,the
choroidplexusepithelialcells,unlikebraincells,aregreatlyenlargedandgranular,consistentwiththeirinabilityto
adequatelytransportfolatefrombloodintocerebrospinalfluid(CSF),andhomovanillicaci
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