EARLY CLINICAL DIAGNOSIS OF CREUTZFELDT-JAKOB DISEASE

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  • FUJITA Kazuhisa
    Department of Medicine, Division of Neurology, Showa University School of Medicine Department of Neurology, Showa University Toyosu Hospital
  • ISHIHARA Kenji
    Department of Medicine, Division of Neurology, Showa University School of Medicine Department of Internal Medicine, Ushioda General Hospital
  • MURAKAMI Hidetomo
    Department of Medicine, Division of Neurology, Showa University School of Medicine
  • KAWAMURA Mitsuru
    Department of Medicine, Division of Neurology, Showa University School of Medicine

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Other Title
  • 剖検例から見たCreutzfeldt-Jakob病の早期診断についての検討
  • ボウケンレイ カラ ミタ Creutzfeldt-Jakobビョウ ノ ソウキ シンダン ニ ツイテ ノ ケントウ

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Abstract

To assess which among the clinical features, electroencephalography (EEG), 14-3-3 protein of cerebrospinal fluid (CSF) and magnetic resonance images (MRI) of the head is useful for early clinical diagnosis of Creutzfeldt-Jakob disease (CJD). We retrospectively analyzed the clinical features, EEG, CSF and MRI of six autopsy-proven cases of CJD. Five cases showed rapidly progressive dementia falling into akinetic mutism at three months. Both myoclonus and periodic sharp wave complexes of EEG were observed in all six cases in the advanced stage. Both CSF 14-3-3 protein and abnormal high signal on diffusion-weighted (DW) MRI were observed in all six cases; the latter was observed in the initial stage, earlier than the former. DW-MRI is useful for early clinical diagnosis of CJD.

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