open access

https://hdl.handle.net/2115/97323

Amyloid-beta pathology in a case with dementia with Lewy bodies with a rapidly progressive clinical course similar to Creutzfeldt-Jacob disease

英語

Rapidly progressive dementia

2024-12-01

John Wiley & Sons

Neuropathology

45(3)

241-247

Most cases of dementia with Lewy bodies (DLB) follow a chronic course. However, some cases of rapidly progressive dementia (RPD) are difficult to distinguish from other diseases. Herein, we report how to differentiate DLB presenting with RPD from other diseases and its pathological features, with examples from our own experience. A 70-year-old man with RPD and psychiatric symptoms, including hallucinations and delusions, was transferred to our hospital. We suspected Creutzfeldt–Jakob disease (CJD), but disease-specific tests were all negative. The patient was treated with corticosteroids on the suspicion of an autoimmune condition; however, his symptoms did not improve. Based on the results of nuclear medicine and other tests, we suspected DLB and administered anti-Parkinsonian drugs; however, they were ineffective, and the patient died. Brain autopsy revealed extensive deposits of Lewy bodies, which were pathologically diagnosed as DLB. Additionally, extensive deposition of senile plaques was observed; however, neurofibrillary tangles (NFTs) were not prominent. DLB generally presents as a chronic disease. However, some patients with DLB present with RPD; therefore, the differential diagnosis of other diseases, such as CJD, is very important. In addition, although this case was not diagnosed with Alzheimer's disease (AD) due to the lack of NFTs, extensive amyloid deposition was observed in the brain tissue. Previous reports have described cases of RPD with amyloid deposition alone, and in this case too, it is suggested that amyloid deposition might have had a strong influence on the clinical course of RPD.

学術雑誌論文

Accepted Manuscript

This is the peer reviewed version of the following article: Fujii, S., Takahashi-Iwata, I., Oshima, Y., Horiuchi, K., Tanei, Z., Satoh, K., Kitamoto, T., Tanaka, S. and Yabe, I. (2025), Amyloid-beta pathology in a case with dementia with Lewy bodies with a rapidly progressive clinical course similar to Creutzfeldt–Jacob disease. Neuropathology., which has been published in final form at https://doi.org/10.1111/neup.13017. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited.

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