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dc.contributor.authorDEMIRKOL, Demet
dc.contributor.authorAlkan, ALPAY
dc.contributor.authorToprak, HÜSEYİN
dc.contributor.authorKILICARSLAN, Rukiye
dc.contributor.authorSharifov, RASUL
dc.date.accessioned2019-10-05T13:17:36Z
dc.date.available2019-10-05T13:17:36Z
dc.date.issued2012-07-01
dc.identifier10.1017/s1041610212001780
dc.identifier.urihttps://hdl.handle.net/20.500.12645/1847
dc.description.abstractMaple syrup urine disease (MSUD) is caused by a genetic defect of branched-chain amino acids, which include leucine, isoleucine and valine. We report diffusion-weighted imaging (DWI) findings in a newborn child with MSUD who presented with acute metabolic encephalopathic crisis. DWI (b = 1,000 s/mm(2)) showed high signal localized within the myelinated white matter (WM) areas including the cerebellar white matter, pons, bulbus, cerebral peduncles, lentiform nucleus, posterior limbs of the internal capsules, corona radiata and bilateral perirolandic cortex. The apparent diffusion coefficient values of these regions were markedly low in the affected areas. The presence of these findings was considered cytotoxic or intramyelinic edema evidenced by restricted water diffusion. In conclusion, our findings suggest that during the acute phase and early encephalopathic crisis stage of MSUD, DWI can demonstrate the involvement of myelinated WM in newborns.
dc.language.isoen
dc.subjectKILICARSLAN R., Alkan A., DEMIRKOL D., Toprak H., Sharifov R., -Maple syrup urine disease: diffusion-weighted MRI findings during acute metabolic encephalopathic crisis-, JAPANESE JOURNAL OF RADIOLOGY, cilt.30, ss.522-525, 2012
dc.titleMaple syrup urine disease: diffusion-weighted MRI findings during acute metabolic encephalopathic crisis
dc.typeArticle
local.avesis.response1717
local.article.journalnameInternational psychogeriatrics
local.org.facultyTıp Fakültesi
dc.identifier.wosWOS:000307289000009
dc.identifier.scopus84866026800
dc.identifier.doi10.1007/s11604-012-0079-2
dc.identifier.pubmed22476847
local.publication.isinternational1


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