Heidenhain Variant of Creutzfeldt-Jakob Disease: Diffusion-Weighted MRI and PET Characteristics

Yoshihisa Tsuji, Hiroshi Kanamori, Gaku Murakami, Masayuki Yokode, Takahiro Mezaki, Katsumi Doh-Ura, Ken Taniguchi, Kozo Matsubayashi, Hidenao Fukuyama, Toru Kita, Makoto Tanaka

Research output: Contribution to journalArticlepeer-review

29 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)63-66
Number of pages4
JournalJournal of Neuroimaging
Issue number1
Publication statusPublished - 2004 Jan
Externally publishedYes


  • 14-3-3 protein
  • Creutzfeldt-Jakob disease
  • Diffusion-weighted MRI
  • PET
  • Visual disturbance
  • Visual evoked potentials

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology


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