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Heidenhain Variant of Creutzfeldtjakob Disease: Diffusion‐Weighted MRI and PET Characteristics
Author(s) -
Tsuji Yoshihisa,
Kanamori Hiroshi,
Murakami Gaku,
Yokode Masayuki,
Mezaki Takahiro,
DohUra Katsumi,
Taniguchi Ken,
Matsubayashi Kozo,
Fukuyama Hidenao,
Kita Toru,
Tanaka Makoto
Publication year - 2004
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/j.1552-6569.2004.tb00218.x
Subject(s) - medicine , diffusion mri , radiology , nuclear medicine , magnetic resonance imaging
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 N75/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 PET in combination with visual evoked potential recording and 14‐3‐3 protein detection may be useful for the early diagnosis of CJD.