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001-es BibID:	BIBFORM063675
Első szerző:	Berghoff, Anna S.
Cím:	Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease / Anna S. Berghoff, Anita Trummert, Ursula Unterberger, Thomas Ströbel, Tibor Hortobágyi, Gabor G. Kovacs
Dátum:	2015
ISSN:	0919-6544
Megjegyzések:	We describe an atypical neuropathological phenotype ofsporadic Creutzfeldt-Jakob disease in a 76-year-old man.The clinical symptoms were characterized by progressivedementia, gait ataxia, rigidity and urinary incontinence.Thedisease duration was 6 weeks.MRI did not show prominentatrophy or hyperintensities in cortical areas, striatum orthalamus.Biomarker examination of the cerebrospinal fluiddeviated from that seen in pure Alzheimer's disease.Triphasic waves in the EEG were detected only later in thedisease course, while 14-3-3 assay was positive. PRNPgenotyping revealed methionine homozygosity (MM) atcodon 129. Neuropathology showed classical CJD changescorresponding to the MM type 1 cases. However, a strikingfeature was the presence of abundant kuru-type plaques inthe white matter. This rare morphology was associated withneuropathological signs of intranuclear inclusion bodydisease and advanced stage of argyrophilic grain disease.These alterations did not show correlation with each other,thus seemed to develop independently. This case furtherhighlights the complexity of neuropathological alterationsin the ageing brain.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban argyrophilic grain disease Creutzfeldt-Jakob disease intranuclear inclusion body disease kuru plaque prion
Megjelenés:	Neuropathology. - 35 : 4 (2015), p. 336-342. -
További szerzők:	Trummert, Anita Unterberger, Ursula Ströbel, Thomas Hortobágyi Tibor (1965-) (patológus) Kovács Gábor Géza (1969-) (neurológus)
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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