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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 
 
 
 
 

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.[1]

References

  1. Heidenhain variant of Creutzfeldt-Jakob disease: diffusion-weighted MRI and PET characteristics. Tsuji, Y., Kanamori, H., Murakami, G., Yokode, M., Mezaki, T., Doh-ura, K., Taniguchi, K., Matsubayashi, K., Fukuyama, H., Kita, T., Tanaka, M. Journal of neuroimaging : official journal of the American Society of Neuroimaging. (2004) [Pubmed]
 
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