Heidenhain variant of Creutzfeldt-Jakob disease: diffusion-weighted MRI and PET characteristics.
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.
Tsuji
Yoshihisa
Y
Department of Geriatric Medicine, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
Kanamori
Hiroshi
H
Murakami
Gaku
G
Yokode
Masayuki
M
Mezaki
Takahiro
T
Doh-ura
Katsumi
K
Taniguchi
Ken
K
Matsubayashi
Kozo
K
Fukuyama
Hidenao
H
Kita
Toru
T
Tanaka
Makoto
M
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