NR APWC
AU Tsuji,Y.; Kanamori,H.; Murakami,G.; Yokode,M.; Mezaki,T.; Doh-ura,K.; Taniguchi,K.; Matsubayashi,K.; Fukuyama,H.; Kita,T.; Tanaka,M.
TI Heidenhain variant of Creutzfeldt-Jakob disease: diffusion-weighted MRI and PET characteristics.
QU Journal of Neuroimaging 2004 Jan; 14(1): 63-6
PT case reports; journal article
AB 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.
MH Brain/pathology/radionuclide imaging; Creutzfeldt-Jakob Syndrome/*diagnosis/radionuclide imaging; *Diffusion Magnetic Resonance Imaging; Evoked Potentials, Visual; Female; Human; Middle Aged; *Tomography, Emission-Computed
AD Department of Geriatric Medicine, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
SP englisch
PO USA