Clinical Neurology最新文献

{"title":"[Clinical practice and research of chronic inflammatory demyelinating polyneuropathy: current status and future].","authors":"Satoshi Kuwabara, Mamoru Doi, Mami Ukai, Haruki Koike, Sonoko Misawa","doi":"10.5692/clinicalneurol.cn-002296","DOIUrl":"10.5692/clinicalneurol.cn-002296","url":null,"abstract":"<p><p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic immune-mediated neuropathy. Japanese national survey conducted in 2021 showed that the estimated number of CIDP patients is 4,180. Whereas CIDP is a rare disease, immunological therapy is highly effective, and therefore appropriate diagnosis and treatment have a substantial clinical impact. In international (2021) and Japanese (2024) clinical practice guidelines, CIDP is not considered a single disease entity but is categorized into typical CIDP and CIDP variant; typical CIDP constitutes the major phenotype, and several distinct variants such as multifocal, distal, and pure motor/sensory CIDP, referred to as CIDP variants. In this review article, we describe current insights into the pathophysiology of CIDP, focusing on typical CIDP, based on findings in electrophysiology, pathology, neuroimaging, and immunology. Current evidence suggests that typical CIDP is antibody-mediated, whereas multifocal CIDP is largely driven by cellular immunity. Previous clinical trials have mainly targeted typical CIDP, and first-line treatments include corticosteroids and immunoglobulin therapy. More recently, the efficacy of the neonatal Fc receptor blocker with a single mechanism of reducing serum IgG levels, supports the concept that typical CIDP is an antibody-mediated disorder. Whereas detailed data on the long-term prognosis of CIDP remain limited, a patient survey conducted by the CIDP Support Group in 2024 has shown that 33% of patients were receiving oral corticosteroid therapy and 57% were undergoing maintenance immunoglobulin therapy. The survey also reported that 69% of patients had to retire or change occupations because of CIDP, suggesting that the impact of treatment on quality of life remains insufficient. A CIDP registry launched in 2022 in Japan has enrolled over 330 patients to date, and real-world investigations of clinical practice are underway. This article provides an overview of the current status, future directions, and remaining challenges in CIDP management and research.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"604-619"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148833539","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":"[Cervical cancer metastasis to the lumbosacral spinal root: a case report].","authors":"Chizuko Sugita, Yoshitsugu Nakamura, Yukiyo Yoshimoto, Satoe Fujiwara, Takafumi Hosokawa, Shigeki Arawaka","doi":"10.5692/clinicalneurol.cn-002272","DOIUrl":"10.5692/clinicalneurol.cn-002272","url":null,"abstract":"<p><p>A 67-year-old woman underwent total hysterectomy for cervical cancer followed by chemotherapy, achieving partial remission. Post-treatment, lymph node metastases to the left obturator region remained decreased in size compared with pre-treatment. Nine months prior to the last chemotherapy, she developed abnormal sensations and muscle weakness in her left lower limb. Nerve conduction studies revealed axonal damage in the left tibial, peroneal, and sural nerves. Pelvic CT and lumbar MRI showed continuous contrast enhancement and enlargement of the left L5, S1, and S2 nerve roots originating from the left lumbosacral plexus. We considered this to be metastasis from the lumbosacral plexus near the lymph node metastasis site of cervical cancer to the lumbosacral nerve roots. When multiple peripheral nerves in the lumbosacral region are involved in patients with cervical cancer, the possibility of direct tumor infiltration into the adjacent nerve plexus followed by progression along axons to the nerve roots must be considered, even when the primary tumor is controlled.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"642-647"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702360","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":"[Microbleed and cerebral hemorrhage due to varicella-zoster virus vasculopathy with contrast enhancement in the anterior choroidal artery: a case report].","authors":"Yasunobu Inagaki, Goro Mitsui, Shinichi Wada, Koji Tsuzaki, Toshiaki Hamano","doi":"10.5692/clinicalneurol.cn-002257","DOIUrl":"10.5692/clinicalneurol.cn-002257","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"652-654"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702350","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":"[Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome with eclampsia, posterior reversible encephalopathy syndrome (PRES), and severe intracerebral hemorrhage in the early postpartum period: a case report].","authors":"Hiroshi Ohara, Yoshihiro Tsukamoto, Ken Kumagai, Masaki Tamura, Kenichi Morita, Aki Sato, Shuichi Igarashi, Masayoshi Tada","doi":"10.5692/clinicalneurol.cn-002202","DOIUrl":"10.5692/clinicalneurol.cn-002202","url":null,"abstract":"<p><p>A 32-year-old woman developed preeclampsia (PE) with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome at 34 weeks of gestation. Despite undergoing emergency cesarean section, adequate blood pressure control, and platelet transfusion, she experienced eclamptic seizures and posterior reversible encephalopathy syndrome (PRES) within 24 hours postpartum, which rapidly progressed to a massive subcortical intracerebral hemorrhage. Multidisciplinary management enabled survival, but she was left with severe neurological sequelae. This case highlights the complex pathological interplay in which endothelial dysfunction associated with PE and HELLP syndrome may precipitate eclampsia and PRES, trigger thrombotic microangiopathy, and ultimately result in catastrophic intracranial hemorrhage. In pregnant women with preeclampsia complicated by HELLP syndrome, early recognition of overlapping manifestations and the risk of postpartum deterioration is essential, underscoring the need for comprehensive multidisciplinary care.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"629-635"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702347","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":"[Ketogenic diet reintroduction in an adult patient with glucose transporter type 1 deficiency syndrome].","authors":"Satoki Kusakabe, Yosuke Osakada, Ryuta Morihara, Yumi Dohi, Kuriko Shimono, Hiroyuki Ishiura","doi":"10.5692/clinicalneurol.cn-002255","DOIUrl":"10.5692/clinicalneurol.cn-002255","url":null,"abstract":"<p><p>Glucose transporter type 1 deficiency syndrome is a metabolic encephalopathy caused by impaired glucose transport across the blood-brain barrier, and ketogenic diet therapy is the first-line treatment. The patient was a 21-year-old woman. In childhood, she developed postexercise hemiparesis and decreased activity during the fasting period. Cerebrospinal fluid testing revealed low glucose levels. Subsequent genetic testing identified a pathogenic SLC2A1 variant, leading to the diagnosis. Her symptoms improved with ketogenic diet therapy. After discontinuation at 18 years of age, she remained stable. However, fasting-related fatigue recurred at the age of 20 years. At 21 years of age, she developed brief (~20 s) episodes of impaired awareness with decreased muscle tone. The EEG showed 3-4 Hz generalized spike-and-wave complex. Ketogenic diet therapy was reintroduced with a ketogenic ratio of 1.5:1, leading to reduced EEG abnormalities and symptomatic improvement. Although symptoms usually lessen in adulthood, the reintroduction of ketogenic diet therapy should be considered when symptoms relapse after adolescence.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"636-641"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702393","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 thiamine deficiency neuropathy with acute progression mimicking Guillain-Barré syndrome].","authors":"Masako Fujita, Chieko Suzuki, Takayasu Utsugisawa, Haruo Nishijima, Masahiko Tomiyama","doi":"10.5692/clinicalneurol.cn-002282","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002282","url":null,"abstract":"<p><p>A 64-year-old man living alone with markedly unbalanced dietary habits presented to the prior hospital with distal sensory disturbances and weakness in all four extremities. After admission, he experienced a rapid progression of lower limb weakness, rendering him unable to walk, for which he was subsequently transferred to our department. During his stay at the previous hospital, he had received lactated Ringer's solution. Laboratory findings revealed a profound deficiency in serum thiamine (vitamin B₁). Thiamine supplementation resulted in rapid clinical improvement, confirming a diagnosis of vitamin B₁ deficiency neuropathy. Although this condition typically follows a subacute course, it can occasionally present acutely, mimicking Guillain-Barré syndrome (GBS), as seen in this case. Our findings suggest that even glucose-free infusions may exacerbate latent vitamin B₁ deficiency by increasing metabolic demand through lactate metabolism and enhancing urinary excretion via diuresis. It is essential to consider vitamin B₁ deficiency in the differential diagnosis of patients presenting with an acute progressive clinical course resembling GBS.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031495","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 cerebral lipiodol embolism presenting with characteristic computed tomography (CT) findings after transcatheter arterial chemoembolization].","authors":"Shumpei Matsumoto, Yoshiki Okamoto, Amane Araki, Kazuhiro Hara, Keizo Yasui","doi":"10.5692/clinicalneurol.cn-002286","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002286","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031528","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 IgG4-related pia mater inflammation].","authors":"Ryoji Naganuma, Shiori Ando, Michinari Okamoto, Takeru Kishinami, Yoshitaka Oda, Ryuji Miyagishi","doi":"10.5692/clinicalneurol.cn-002284","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002284","url":null,"abstract":"<p><p>A 74-year-old man presented with slowly progressive ataxia and impaired consciousness. Brain MRI demonstrated hyperintensity on diffusion-weighted imaging (DWI) in the bilateral frontal subdural spaces and cerebral sulci, mild FLAIR hyperintensity in the subarachnoid space, and gadolinium enhancement along the pia mater of the left frontal cortex, suggesting meningitis. Cerebrospinal fluid analysis revealed pleocytosis with elevated protein but normal glucose, and aseptic meningitis was suspected based on the clinical course and laboratory findings. Because his serum IgG4 level was elevated, IgG4-related disease was considered as an underlying etiology. Brain biopsy showed inflammatory cell infiltration in the dura mater, arachnoid mater, and cortical surface, while clusters of CD138-positive plasma cells were observed only in the pia mater adjacent to the brain parenchyma. Immunostaining revealed that more than 40% of IgG-positive plasma cells were IgG4-positive, with approximately 100 IgG4-positive cells per high-power field, without κ/λ light-chain restriction. Although storiform fibrosis and obliterative phlebitis were absent, the findings were compatible with IgG4-related disease in combination with the clinical presentation. The patient was treated with intravenous methylprednisolone followed by oral prednisolone, resulting in rapid improvement of consciousness and reduction of leptomeningeal enhancement on MRI. This case represents a rare form of IgG4-related leptomeningitis in which the pia mater is the primary site of involvement. The findings underscore the importance of considering IgG4-related disease in the differential diagnosis of aseptic meningitis, particularly when imaging suggests pia mater inflammation and serum IgG4 levels are elevated.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031514","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 Parkinsonism possibly associated with long-term methamphetamine use].","authors":"Yasuhiro Aso, Teruaki Masuda, Kaori Sumi, Katsunori Takahata, Noriyuki Kimura","doi":"10.5692/clinicalneurol.cn-002266","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002266","url":null,"abstract":"<p><p>We report the case of a 60-year-old male who developed parkinsonism following approximately 32 years of methamphetamine (MA) use. The patient had used MA since age 18. He initially noted a mild resting tremor in his right upper limb around age 50, which remained relatively stable for nearly a decade. However, at age 59, gait disturbances emerged and progressed over the following six months. While brain magnetic resonance imaging (MRI) showed no remarkable findings, 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy demonstrated a reduction in the heart-to-mediastinum ratio. Additionally, dopamine transporter single-photon emission computed tomography (DAT-SPECT) revealed decreased uptake in the bilateral striatum. Treatment with levodopa resulted in significant clinical improvement. Given that MA is known to induce neurotoxicity in dopaminergic neurons through mechanisms such as oxidative stress and α-synuclein aggregation, these findings suggest that prolonged MA exposure may trigger a neurodegenerative process leading to parkinsonism with pathophysiological features similar to Parkinson's disease.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031468","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":"[How to deal with peer review effectively for case reports].","authors":"Akiyuki Hiraga","doi":"10.5692/clinicalneurol.cn-002299","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002299","url":null,"abstract":"<p><p>To achieve acceptance of a case report, authors must provide an effective response to the reviewers' comments and perform a thorough revision of the manuscript. Authors should adopt three key mindsets: (i) reviewers are not adversaries, but collaborators who help improve the manuscript; (ii) if a reviewer misunderstands a point, the writing should be clarified; and (iii) the revised manuscript should be resubmitted promptly. When responding to reviewers, authors should follow three fundamental principles: (i) address all comments, (ii) respond politely, and (iii) provide evidence when offering a rebuttal. The final decision to accept or reject a paper is made by the editors, not the reviewers. Therefore, when responding to a comment is difficult, statements such as \"we shall abide by the editor's decision\" in the cover letter may be effective.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149031482","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}