Clinical Neurology最新文献

{"title":"[Autoimmune limbic encephalitis with concurrent positivity for Caspr2 and GFAPα antibodies].","authors":"Akiko Hamamoto, Tomohito Nakano, Hisae Sumi, Takashi Naka, Akio Kimura, Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-002250","DOIUrl":"10.5692/clinicalneurol.cn-002250","url":null,"abstract":"<p><p>A 69-year-old man presented with progressive cognitive decline over three months and two episodes of status epilepticus. Brain MRI revealed abnormal signals in the bilateral medial temporal lobes and deep white matter, and brain perfusion scintigraphy demonstrated increased perfusion in these regions. Electroencephalography showed sharp and slow-wave complexes with an F8 focus. Both serum and cerebrospinal fluid were positive for contactin-associated protein 2 (Caspr2) antibodies, and cerebrospinal fluid was positive for glial fibrillary acidic protein α (GFAPα) antibodies. Immunotherapy improved the clinical symptoms and MRI abnormalities, including deep white matter lesions. White matter involvement is rare in Caspr2 antibody-positive limbic encephalitis, and in this case, GFAPα antibodies may have contributed to the white matter lesions.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"548-552"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of pure autonomic failure with a long-term-45-year-clinical course since the initial detection of abnormal blood pressure fluctuation.","authors":"Tetsuro Yanagisawa, Amane Araki, Kazuhiro Hara, Saori Morozumi, Keizo Yasui","doi":"10.5692/clinicalneurol.cn-002226","DOIUrl":"10.5692/clinicalneurol.cn-002226","url":null,"abstract":"<p><p>This report describes the case of an 85-year-old male with a clinical phenotype consistent with pure autonomic failure (PAF) for more than four decades. Despite the presence of neurological manifestations, including bradykinesia, olfactory dysfunction, and visual hallucinations, phenoconversion to another α-synucleinopathy has not been confirmed based on established diagnostic criteria. Orthostatic and postprandial hypotension, low supine plasma norepinephrine levels, and reduced cardiac uptake on ¹²³I-metaiodobenzylguanidine myocardial scintigraphy were observed, consistent with the postganglionic cardiac sympathetic denervation frequently observed in Lewy body disease. Given the extraordinarily prolonged disease course and early central neurological signs, this condition may represent a preclinical stage of Parkinson's disease or dementia with Lewy bodies. This case provides insight into the natural history of PAF and its clinical variability within the Lewy body disease spectrum.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"529-534"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-NF155 antibody-positive autoimmune nodopathy with myocardial 123I-MIBG scintigraphy abnormality.","authors":"Suguru Kadowaki, Akeshi Morimatsu, Akiko Shirata, Kiyomi Yamane","doi":"10.5692/clinicalneurol.cn-002212","DOIUrl":"10.5692/clinicalneurol.cn-002212","url":null,"abstract":"<p><p>A 68-year-old man presented with numbness and pain of the hands. Neurological examination revealed distal limb weakness, diminished tendon reflexes, sensory disturbances in all extremities, tremors, ataxia, and orthostatic hypotension, which caused repeated fainting spells and falls. The nerve conduction studies revealed definite demyelinating abnormalities. The cerebrospinal fluid protein levels were markedly increased at 638 mg/dl. The contrast-enhanced MRI neurography revealed nerve root enlargement and enhancement, and the 123I-metaiodobenzylguanidine myocardial scintigraphy revealed abnormalities, indicating postganglionic sympathetic small fiber disturbance. This patient was initially diagnosed with a distal acquired demyelinating symmetric type of chronic idiopathic demyelinating polyradiculoneuropathy (CIDP). Intravenous immunoglobulin (IVIg) therapy was nearly ineffective, which was different from typical CIDP, and corticosteroids demonstrated mild efficacy. Five years after his initial visit, anti-neurofascin-155 (NF155) autoimmune nodopathy was suspected based on his pathognomonic symptoms, examination results, and ineffectiveness of IVIg. Anti-NF155-IgG4 antibodies were negative; however, anti-NF155-IgG antibodies tested positive, leading to a final diagnosis of anti-NF155 antibody-positive autoimmune nodopathy. This case highlights the clinical significance of non-IgG4 class anti-NF155-IgG antibodies and the involvement of postganglionic sympathetic small fibers in anti-NF155-IgG-related autoimmune nodopathy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"473-478"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148253962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Word fluency for \"Ka\" in Parkinson's disease patients: comparison with memory clinic patients].","authors":"Mina Yasugi, Noriyuki Miyaue, Hayato Yabe","doi":"10.5692/clinicalneurol.cn-002241","DOIUrl":"10.5692/clinicalneurol.cn-002241","url":null,"abstract":"<p><p>Domestic studies examining word fluency tasks in patients with Parkinson's disease (PD) are limited. This study compared quantitative and qualitative features of a word fluency task included in the Japanese version of the Montreal Cognitive Assessment (MoCA-J) between patients with PD and patients attending a memory clinic. We retrospectively analyzed the MoCA-J word fluency task requiring the generation of words beginning with the Japanese syllable \"ka\" in 100 patients with PD and 100 memory clinic patients. The number of recalled words and linguistic characteristics of the responses were evaluated. Cognitive function was assessed using the Mini-Mental State Examination (MMSE) and MoCA-J. The mean MMSE scores were higher in the PD group than in the memory clinic group (27.6 vs. 24.4), as were MoCA-J scores (23.5 vs. 18.7). The mean number of recalled words did not differ between groups (PD: 9.1; memory clinic: 9.2) and showed positive correlations with cognitive scores. The PD group produced 318 unique words, whereas the memory clinic group produced 285. The proportion of adjectives was lower in the PD group. No significant between-group differences were observed in semantic category classifications, including object names, motion-related words, and emotion/state-related words. Although global cognitive function was relatively preserved in patients with PD, their word fluency performance was comparable to that of memory clinic patients, with subtle differences in linguistic characteristics.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"466-472"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148253975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survey on awareness of sleep medicine among members of the Japanese Society of Neurology.","authors":"Hiroaki Fujita, Keisuke Kido, Naohiro Egawa, Naoko Tachibana, Makoto Kawai, Masayuki Miyamoto, Tomoyuki Miyamoto, Takuya Oguri, Ariyuki Hori, Yasunori Oka, Takashi Nomura, Hiroshi Chatani, Keisuke Suzuki","doi":"10.5692/clinicalneurol.cn-002245","DOIUrl":"10.5692/clinicalneurol.cn-002245","url":null,"abstract":"<p><strong>Background: </strong>Sleep-wake problems and sleep-related disorders, which are common attributes of neurological diseases, often remain under-recognized and under-treated. Insufficient education in sleep medicine might contribute to this gap.</p><p><strong>Methods: </strong>The Sleep Medicine Section of the Japanese Society of Neurology conducted a nationwide, web-based survey of its full-member physicians (N = 9,703), assessing neurologists' education, clinical practice, and interest in sleep medicine.</p><p><strong>Results: </strong>Of the 904 survey participants, only 10% considered their undergraduate education on sleep-wake problems and sleep-related disorders as adequate, whereas 86% rated it as insufficient. Furthermore, nearly 90% of the participants recognized the importance of these education gaps in neurological practice, and more than 90% expressed a desire for further education. Regarding clinical management, fewer than 5% felt confident and 15% expressed anxiety or a tendency to avoid such cases. Approximately 87% reported encountering such problems and disorders in clinical practice, and 47% reported managing them at least weekly. Insomnia, circadian rhythm sleep-wake disorders, and hypersomnia were considered the most difficult to treat. Respondents showed strong interest in lectures and on-demand webinars on rapid eye movement sleep behavior disorder (71.1%), restless legs syndrome (62.3%), and the relationship between sleep-wake problems or sleep-related disorders and neurodegenerative diseases (77.1%) or dementia (68.3%).</p><p><strong>Conclusions: </strong>Although the importance of sleep-wake problems and sleep-related disorders and additional educational opportunities are well-recognized, confidence in diagnosis and treatment remained low. This survey revealed an unmet clinical need in sleep medicine among Japanese neurologists, underscoring the importance of structured, accessible education at undergraduate and postgraduate levels.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"457-465"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148254005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Metagenomic analysis for central nervous system infections: clinical utility and future directions].","authors":"Yusuke Sakiyama","doi":"10.5692/clinicalneurol.cn-002248","DOIUrl":"10.5692/clinicalneurol.cn-002248","url":null,"abstract":"<p><p>Encephalitis and meningitis are neurological emergencies in which delayed diagnosis may lead to severe neurological sequelae, necessitating accurate and rapid etiological identification. In recent years, metagenomic next-generation sequencing (mNGS), which enables comprehensive analysis of microbial genomes without prespecified hypotheses, has attracted increasing attention. Its clinical application in neuroinfectious diseases has contributed to improved diagnostic yield and the detection of rare pathogens. In particular, mNGS has been shown to be useful in clinically challenging situations such as culture-negative cases, anaerobic infections, mixed infections, and immunocompromised hosts. However, the technology also has inherent limitations, including enormous data volume, challenges in interpreting pathogenic relevance, limited turnaround time, high cost, and a lack of standardized analytical pipelines. Thus, although mNGS represents a valuable complementary tool to conventional diagnostic methods, it is not universally applicable, and its results must be carefully interpreted within appropriate clinical contexts.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"443-449"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148273220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Survey on the involvement of Japanese neurologists in genetic medicine].","authors":"Yuka Shibata, Hyangri Chang, Shinichiro Yamada, Masaaki Matsushima, Kazumasa Saigoh, Hiroyuki Ishiura, Yoshiki Sekijima, Hirofumi Maruyama, Takeshi Ikeuchi, Kazuko Hasegawa, Masashi Aoki, Masahisa Katsuno, Tatsushi Toda, Ichiro Yabe","doi":"10.5692/clinicalneurol.cn-002237","DOIUrl":"10.5692/clinicalneurol.cn-002237","url":null,"abstract":"<p><p>With the advancement of genetic medicine, opportunities to perform genetic testing in neurology practice are increasing. To address this trend, we conducted a survey on the involvement of neurologists in genetic medicine. A total of 658 respondents (9.6%) participated, and 94.4% reported experience in performing genetic testing. The increased opportunity to perform genetic testing was attributed to the recent emergence of disease-modifying therapies, which are beneficial for the early diagnosis and treatment of patients, as well as the growing need to consider reproductive options such as prenatal testing and preimplantation genetic testing for monogenic disorders. Educational opportunities aimed at improving the genetic medicine skills of all neurologists are urgently needed.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"450-456"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148273302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A case of anti-Kelch-like protein 11 (KLHL11) antibody-positive opsoclonus-myoclonus syndrome associated with small cell lung cancer that improved after immunotherapy and cancer treatment].","authors":"Riho Takai, Marina Saito, Akifumi Mitsuishi, Manami Maruko, Yuki Kujuro, Hidenori Hattori","doi":"10.5692/clinicalneurol.cn-002240","DOIUrl":"10.5692/clinicalneurol.cn-002240","url":null,"abstract":"<p><p>Opsoclonus-myoclonus syndrome (OMS), characterized by symptoms including rapid eye movements, myoclonus, and cerebellar ataxia, is one of the paraneoplastic neurological syndrome. A 68-year-old woman was admitted with oscillopsia, dizziness, and gait disturbance caused by postural tremor. Clinical evaluation led to a diagnosis of Kelch-like protein 11 (KLHL11)-associated paraneoplastic OMS, secondary to small cell lung cancer (cT1cN3M1a, stage 4A). In most cases, the improvement of the neurological symptoms is limited unless the underlying malignancy is effectively treated. Therefore, cancer therapy in combination with immunotherapy is essential, even in patients with poor performance status. Here, we report a case in which both immunotherapy and cancer treatment led to improvement in neurological symptoms associated with KLHL11-related paraneoplastic OMS.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"485-491"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148253951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A case of Takayasu arteritis diagnosed following posterior reversible encephalopathy syndrome].","authors":"Ryutaro Otsu, Mai Iwahara, Kohei Miyagawa, Yasuhisa Akaiwa, Toshiki Nakamura, Tomoyuki Miyamoto","doi":"10.5692/clinicalneurol.cn-002228","DOIUrl":"10.5692/clinicalneurol.cn-002228","url":null,"abstract":"<p><p>A 53-year-old woman presented with chronic anorexia and gait disturbance. During evaluation at another hospital, brain MRI revealed abnormal high signal intensities in both supratentorial and infratentorial regions, prompting referral and admission to our hospital. On admission, she exhibited non-lateralized hypertension, decreased responsiveness, hypophonia, masked face, bradykinesia, shuffling gait, and hyperreflexia. Blood tests showed mild inflammatory markers. Based on her hypertension and MRI findings, posterior reversible encephalopathy syndrome (PRES) was diagnosed, and antihypertensive therapy was initiated. Her symptoms and imaging findings improved, though some abnormalities persisted. During further evaluation for the cause of hypertension, she contracted COVID-19. Despite recovery, low-grade fever and inflammatory response persisted. Contrast-enhanced CT revealed perivascular enhancement around the aortic arch branches, leading to a diagnosis of Takayasu arteritis. This case highlights PRES as a clinical trigger for the diagnosis of Takayasu arteritis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"479-484"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148273280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A case of von Hippel-Lindau disease showing characteristic progressive growth of multiple cerebellar hemangioblastomas].","authors":"Hideki Hayashi, Tomohiko Ishihara, Natsumi Saito, Tetsuya Hiraishi, Osamu Onodera","doi":"10.5692/clinicalneurol.cn-002230","DOIUrl":"10.5692/clinicalneurol.cn-002230","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"492-493"},"PeriodicalIF":0.0,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148254008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}