title: Heidenhain variant of Creutzfeldt-Jakob disease: diffusion-weighted MRI and PET characteristics.
abstract: Creutzfeldt-Jakob disease (CJD) is characterized by rapidly progressive dementia with a variety of neurological disorders and a fatal outcome. The authors present a case with visual disturbance as a leading symptom and rapid deterioration in global cognitive functions. The cerebrospinal fluid was positive for 14-3-3 protein, and diffusion-weighted magnetic resonance imaging (MRI) showed marked hyperintensity in the parieto-occipital cortices, where hypometabolism was clearly detected on positron emission tomography (PET). Pattern-reversal visual evoked potentials showed prolonged P100 latencies and increased N/5/P100 amplitudes. All these findings supported a diagnosis of the Heidenhain variant of CJD, whereas a long clinical course, a lack of myoclonus, and an absence of periodic synchronous discharges on electroencephalography were atypical. Diffusion-weighted MRI and PE1 in combination with visual evoked potential recording and 14-3-3 protein detection may be useful for the early diagnosis of CJD.
authors.affiliation: Department of Geriatric Medicine, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Tsuji
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kanamori
authors.forename: Gaku
authors.initials: G
authors.lastname: Murakami
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yokode
authors.forename: Takahiro
authors.initials: T
authors.lastname: Mezaki
authors.forename: Katsumi
authors.initials: K
authors.lastname: Doh-ura
authors.forename: Ken
authors.initials: K
authors.lastname: Taniguchi
authors.forename: Kozo
authors.initials: K
authors.lastname: Matsubayashi
authors.forename: Hidenao
authors.initials: H
authors.lastname: Fukuyama
authors.forename: Toru
authors.initials: T
authors.lastname: Kita
authors.forename: Makoto
authors.initials: M
authors.lastname: Tanaka
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D038524:Diffusion Magnetic Resonance Imaging; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D008875:Middle Aged; D014055:Tomography, Emission-Computed