| Title: | MM2 cortical form of sporadic Creutzfeldt-Jakob disease without progressive dementia and akinetic mutism : A case deviating from current diagnostic criteria |
| Authors: | Takahashi-Iwata, Ikuko Browse this author |
| Yabe, Ichiro Browse this author →KAKEN DB |
| Kudo, Akihiko Browse this author |
| Eguchi, Katsuya Browse this author |
| Wakita, Masahiro Browse this author |
| Shirai, Shinichi Browse this author |
| Matsushima, Masaaki Browse this author |
| Toyoshima, Takanobu Browse this author |
| Chiba, Susumu Browse this author |
| Tanikawa, Satoshi Browse this author →KAKEN DB |
| Tanaka, Shinya Browse this author →KAKEN DB |
| Satoh, Katsuya Browse this author →KAKEN DB |
| Kitamoto, Tetsuyuki Browse this author →KAKEN DB |
| Sasaki, Hidenao Browse this author →KAKEN DB |
| Keywords: | Sporadic Creutzfeldt-Jakob disease |
| Corticobasal syndrome |
| MM2-cortical form |
| Issue Date: | 15-May-2020 |
| Publisher: | Elsevier |
| Journal Title: | Journal of the neurological sciences |
| Volume: | 412 |
| Start Page: | 116759 |
| Abstract: | Sporadic Creutzfeldt-Jakob disease (sCJD) is classified into six types based on codon 129 polymorphism in the PRNP gene and the protease-resistant prion-related protein, PrP [1,2]. This classification corresponds well with the clinical course and the pathological findings. MM2-cortical type sCJD (MM2C-sCJD) is clinically characterized by slow progressive dementia, increased levels of 14-3-3 protein in the cerebrospinal fluid (CSF), and no periodic synchronous discharge (PSD) in electroencephalography [3]. We report the case of a patient presented with chronic progressive cortical symptoms. Based on the initial clinical findings, corticobasal syndrome (CBS) was suspected although he did not develop akinetic mutism during the lifetime. The postmortem pathological and anatomical findings confirmed MM2C-sCJD. As per the existing diagnostic criteria, our case was difficult to diagnose during the patient’s lifetime. Therefore, this is an important case for considering future revisions of the diagnostic criteria for MM2C-sCJD. |
| Rights: | © 2020, Elsevier. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ |
| http://creativecommons.org/licenses/by-nc-nd/4.0/ |
| Type: | article (author version) |
| URI: | http://hdl.handle.net/2115/81396 |
| Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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