{"title":"日本皮质基底层变性患者的临床特征。","authors":"","doi":"10.1016/j.jns.2024.123212","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p>Corticobasal degeneration (CBD) is a clinically heterogeneous neurodegenerative disorder, for which pathological investigations are essential for a definitive diagnosis. This study explored the clinical characteristics of Japanese patients with pathologically confirmed CBD.</p></div><div><h3>Methods</h3><p>We reviewed the data of Japanese patients with pathologically confirmed CBD who were consecutively autopsied at our institute. Clinical data were obtained from medical records and clinicopathological conferences.</p></div><div><h3>Results</h3><p>Of the 34 patients initially reviewed, three were excluded because of a lack of detailed clinical data. Of the remaining 31 patients, 16 were men and 15 were women. The mean ages at onset and death were 63.3 ± 6.7 (51–79) years and 69.1 ± 6.9 (54–86), respectively. The median disease duration was 6.0 (2.5–12) years. The clinical phenotypes were as follows: progressive supranuclear palsy syndrome (PSPS; <em>n</em> = 20, 64.5 %), probable or possible corticobasal syndrome (<em>n</em> = 6, 19.4 %), frontal behavioral-spatial syndrome (<em>n</em> = 4, 12.9 %), nonfluent/agrammatic variant of primary progressive aphasia (<em>n</em> = 1, 3.2 %). Furthermore, 28 (90.3 %) patients exhibited dysphagia with a median latency of 3.5 (1.0–10.0) years, and 22 (71.0 %) patients who underwent tube feeding survived longer than those who did not (<em>P</em> = 0.013).</p></div><div><h3>Conclusions</h3><p>Compared with Western populations, a high prevalence of PSPS may be a clinical characteristic of Japanese patients with CBD. Additionally, dysphagia occurs in many patients with early latency and may shorten survival. Tube feeding contributes to the prolonged survival of patients with CBD.</p></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":3.6000,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical characteristics of Japanese patients with corticobasal degeneration\",\"authors\":\"\",\"doi\":\"10.1016/j.jns.2024.123212\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Introduction</h3><p>Corticobasal degeneration (CBD) is a clinically heterogeneous neurodegenerative disorder, for which pathological investigations are essential for a definitive diagnosis. This study explored the clinical characteristics of Japanese patients with pathologically confirmed CBD.</p></div><div><h3>Methods</h3><p>We reviewed the data of Japanese patients with pathologically confirmed CBD who were consecutively autopsied at our institute. Clinical data were obtained from medical records and clinicopathological conferences.</p></div><div><h3>Results</h3><p>Of the 34 patients initially reviewed, three were excluded because of a lack of detailed clinical data. Of the remaining 31 patients, 16 were men and 15 were women. The mean ages at onset and death were 63.3 ± 6.7 (51–79) years and 69.1 ± 6.9 (54–86), respectively. The median disease duration was 6.0 (2.5–12) years. The clinical phenotypes were as follows: progressive supranuclear palsy syndrome (PSPS; <em>n</em> = 20, 64.5 %), probable or possible corticobasal syndrome (<em>n</em> = 6, 19.4 %), frontal behavioral-spatial syndrome (<em>n</em> = 4, 12.9 %), nonfluent/agrammatic variant of primary progressive aphasia (<em>n</em> = 1, 3.2 %). Furthermore, 28 (90.3 %) patients exhibited dysphagia with a median latency of 3.5 (1.0–10.0) years, and 22 (71.0 %) patients who underwent tube feeding survived longer than those who did not (<em>P</em> = 0.013).</p></div><div><h3>Conclusions</h3><p>Compared with Western populations, a high prevalence of PSPS may be a clinical characteristic of Japanese patients with CBD. Additionally, dysphagia occurs in many patients with early latency and may shorten survival. Tube feeding contributes to the prolonged survival of patients with CBD.</p></div>\",\"PeriodicalId\":17417,\"journal\":{\"name\":\"Journal of the Neurological Sciences\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":3.6000,\"publicationDate\":\"2024-09-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of the Neurological Sciences\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0022510X24003472\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Neurological Sciences","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0022510X24003472","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Clinical characteristics of Japanese patients with corticobasal degeneration
Introduction
Corticobasal degeneration (CBD) is a clinically heterogeneous neurodegenerative disorder, for which pathological investigations are essential for a definitive diagnosis. This study explored the clinical characteristics of Japanese patients with pathologically confirmed CBD.
Methods
We reviewed the data of Japanese patients with pathologically confirmed CBD who were consecutively autopsied at our institute. Clinical data were obtained from medical records and clinicopathological conferences.
Results
Of the 34 patients initially reviewed, three were excluded because of a lack of detailed clinical data. Of the remaining 31 patients, 16 were men and 15 were women. The mean ages at onset and death were 63.3 ± 6.7 (51–79) years and 69.1 ± 6.9 (54–86), respectively. The median disease duration was 6.0 (2.5–12) years. The clinical phenotypes were as follows: progressive supranuclear palsy syndrome (PSPS; n = 20, 64.5 %), probable or possible corticobasal syndrome (n = 6, 19.4 %), frontal behavioral-spatial syndrome (n = 4, 12.9 %), nonfluent/agrammatic variant of primary progressive aphasia (n = 1, 3.2 %). Furthermore, 28 (90.3 %) patients exhibited dysphagia with a median latency of 3.5 (1.0–10.0) years, and 22 (71.0 %) patients who underwent tube feeding survived longer than those who did not (P = 0.013).
Conclusions
Compared with Western populations, a high prevalence of PSPS may be a clinical characteristic of Japanese patients with CBD. Additionally, dysphagia occurs in many patients with early latency and may shorten survival. Tube feeding contributes to the prolonged survival of patients with CBD.
期刊介绍:
The Journal of the Neurological Sciences provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. JNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials).
JNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism.