title: Altered basal ganglia echogenicity early in sporadic Creutzfeldt-Jakob disease.
abstract: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease caused by conformational alteration of the ubiquitous prion protein. Sporadic CJD appears to progress faster if the basal ganglia are shown to be affected on magnetic resonance imaging. Transcranial B-mode sonography (TCS) enables visualization of differences in tissue echogenicity, which can be associated with changes in the cerebral metabolism of various metals. These metabolic changes are considered 1 of the potential mechanisms of the brain damage in CJD; TCS hyperechogenicity may reflect changes in metal homeostasis in CJD. We report a 63-year-old woman who presented with typical sporadic CJD. One month after she fell ill, a magnetic resonance imaging scan of her brain showed diffuse cortical but no obvious basal ganglia involvement. However, TCS revealed moderate hyperechogenicity of both lentiform nuclei. The patient's disease progressed quickly and she died 2 months later. TCS may show basal ganglia alteration early in the disease course of patients with quickly progressing CJD, thus aiding in premortem diagnosis.
authors.affiliation: *Clinic for Neurology, Faculty of Medicine ‡Faculty for Special Education and Rehabilitation, University of Belgrade, Belgrade, Serbia †Diagnostic Center VAMED-VMR, Novi Sad, Serbia.
authors.forename: Nikola
authors.initials: N
authors.lastname: Veselinovic
authors.forename: Aleksandra M
authors.initials: AM
authors.lastname: Pavlovic
authors.forename: Boris
authors.initials: B
authors.lastname: Petrovic
authors.forename: Aleksandar
authors.initials: A
authors.lastname: Ristic
authors.forename: Ivana
authors.initials: I
authors.lastname: Novakovic
authors.forename: Tamara
authors.initials: T
authors.lastname: Svabic Medjedovic
authors.forename: Dragan
authors.initials: D
authors.lastname: Pavlovic
authors.forename: Nada
authors.initials: N
authors.lastname: Sternic
mesh_terms: D001479:Basal Ganglia / Q000000981:diagnostic imaging*; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology; D018450:Disease Progression; D004453:Echoencephalography; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008875:Middle Aged