Clinical Neurology最新文献

{"title":"[A case of autoantibody-negative autoimmune encephalitis associated with mature ovarian teratoma, successfully treated with early ovariectomy].","authors":"Kensuke Takahashi, Takuya Sasaki, Reiko Kawasaki, Rena Maeda, Yukio Kakuta, Ichiro Imafuku","doi":"10.5692/clinicalneurol.cn-002169","DOIUrl":"10.5692/clinicalneurol.cn-002169","url":null,"abstract":"<p><p>An 18-year-old woman was admitted to our hospital with altered consciousness and urinary retention. Brain MRI revealed FLAIR hyperintensity along the brainstem surface. Paraneoplastic encephalitis was suspected due to the presence of an ovarian teratoma, and an ovariectomy was performed on the day of admission. Both steroid pulse therapy and high-dose intravenous immunoglobulin therapy were initiated, leading to an improvement in her clinical symptoms and MRI findings. All tested autoantibodies were negative. There have been no reports on the optimal timing of ovariectomy in cases of autoimmune encephalitis associated with ovarian teratoma, except in anti-N-methyl-D-aspartate receptor encephalitis. However, early tumor resection may be desirable for antigen removal, even in cases of autoimmune encephalitis without anti-N-methyl-D-aspartate receptor antibody.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"155-160"},"PeriodicalIF":0.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146203199","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 recurrent cerebellitis leading to the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).","authors":"Yuko Ito, Chikako Ochi, Yuki Yamanishi, Hiroshi Takashima, Akihiro Hashiguchi, Masahiro Nagai","doi":"10.5692/clinicalneurol.cn-002200","DOIUrl":"10.5692/clinicalneurol.cn-002200","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"192-193"},"PeriodicalIF":0.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146143825","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 report: an 89-year-old man taking prednisolone for chronic inflammatory demyelinating polyradiculoneuropathy diagnosed with human herpesvirus 6 variant A encephalitis].","authors":"Sawaka Yoshitake, Bunei Paku, Miho Sakuma, Keiko Ichinose, Saneyuki Mizutani, Mutsufusa Watanabe","doi":"10.5692/clinicalneurol.cn-002209","DOIUrl":"10.5692/clinicalneurol.cn-002209","url":null,"abstract":"<p><p>Human herpesvirus 6 (HHV-6) is a neurotropic virus comprising two variants, HHV-6A and HHV-6B. Although HHV-6B is known to cause exanthem subitum in children, the clinical significance of HHV-6A remains poorly understood, particularly in adult and elderly populations. Here, we report a rare case of HHV-6A encephalitis in an immunosuppressed elderly patient. An 89-year-old male patient with chronic inflammatory demyelinating polyradiculoneuropathy was administered immunosuppressive therapy with prednisolone. His consciousness progressively worsened over 4 days. Brain magnetic resonance imaging with fluid-attenuated inversion recovery demonstrated a hyperintense lesion in the left medial temporal lobe. The FilmArray meningitis/encephalitis panel tested positive for HHV-6. Further testing revealed elevated HHV-6A DNA levels in both the cerebrospinal fluid and plasma, confirming the diagnosis of HHV-6A encephalitis. A moderate increase in HHV-6A DNA and a slow clinical course suggest that chromosomally integrated HHV-6 (ciHHV-6) was unlikely. The treatment consisted of tapering prednisolone and initiating foscarnet, which gradually improved the patient's level of consciousness. This case highlights the importance of HHV-6A in the differential diagnosis of encephalitis, particularly in elderly patients receiving immunosuppressive therapy. Early diagnosis using a multiplex PCR panel, combined with prompt initiation of antiviral treatment, may lead to more favorable outcomes. Further studies are required to clarify the pathogenesis, clinical spectrum, and optimal management of HHV-6A-associated central nervous system diseases.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"177-181"},"PeriodicalIF":0.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146203222","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":"[Clinical support and education in muscle disease-Experiences from Japan and Asian regions].","authors":"Ichizo Nishino","doi":"10.5692/clinicalneurol.cn-002247","DOIUrl":"10.5692/clinicalneurol.cn-002247","url":null,"abstract":"<p><p>The author has been continuously engaged in diagnostic support for muscle disease, specialist education, and international medical support, mainly in Asian regions. In this article, experiences in muscle disease diagnosis in Japan and medical support activities in Asia are shared, and conditions necessary for the development of muscle disease care are considered based on these experiences. Muscle diseases are ultra-rare conditions among rare diseases and require a high level of expertise as well as centralized diagnostic systems for accurate diagnosis and evaluation of disease pathology. The development of muscle disease care depends on several key factors, including the presence of core specialists, subspecialization with effective collaborative frameworks, an adequate number of medical professionals, financial support, economic development, and social stability. Japan is characterized by an environment in which these conditions are largely fulfilled. From the author's perspective, this environment provides an important opportunity to pursue activities that go beyond routine clinical practice, including diagnostic support, education, and international collaboration, thereby contributing to the advancement of muscle disease care.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"151-154"},"PeriodicalIF":0.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146203289","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":"[Advanced imaging diagnosis and therapeutic strategies for ischemic stroke].","authors":"Manabu Inoue","doi":"10.5692/clinicalneurol.cn-002188","DOIUrl":"10.5692/clinicalneurol.cn-002188","url":null,"abstract":"<p><p>In recent years, advances in imaging analysis technologies, including CT perfusion, MRI, and AI analysis, have extended the therapeutic time window for acute ischemic stroke to as long as 24 hours. In Japan, however, while MRI remains the predominant modality, the dissemination and utilization of perfusion imaging are still limited, and institutional challenges persist. Moreover, even among neurologists specializing in stroke, the understanding and integration of perfusion imaging and AI technologies remain insufficient. This article provides an overview of these developments, and it is anticipated that future strategies will focus on constructing novel diagnostic approaches that integrate AI technologies with Japan's unique MRI environment.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"58-66"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019925","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":"Delayed dysgeusia in five patients following MRI-guided focused ultrasound surgery targeting the ventral intermediate nucleus of the ‍thalamus for essential tremor.","authors":"Noriaki Tashiro, Masahiro Yasaka, Yasushi Miyagi","doi":"10.5692/clinicalneurol.cn-002149","DOIUrl":"10.5692/clinicalneurol.cn-002149","url":null,"abstract":"<p><p>Perioperative complications of MR-guided focused ultrasound therapy for Vim-targeted tremor are often reported, such as weakness and paresthesia. In this paper, five cases of dysgeusia after MR-guided focused ultrasound therapy targeting the Vim in patients with essential tremor are presented. All patients also suffered from sensory disturbances. In most cases, dysgeusia is mild, but there have been reports of patients with severe weight loss. Dysgeusia has also been reported in deep brain stimulation therapy. Dysgeusia is a complication that should be considered in the treatment of Vim-targeted tremor. Our own cases of taste disorder are reported, along with a review of the literature.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"74-77"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145985765","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 fat embolism with unfavorable outcome demonstrated by temporal changes on serial brain MRI].","authors":"Tetsuro Yanagisawa, Erika Suzuki, Amane Araki, Saori Morozumi, Keizo Yasui","doi":"10.5692/clinicalneurol.cn-002175","DOIUrl":"10.5692/clinicalneurol.cn-002175","url":null,"abstract":"<p><p>A 74-year-old man sustained a left femoral neck fracture following a fall on day X. Approximately four hours after the injury, he developed impaired consciousness, which persisted even after osteosynthesis was performed on day X+1. Brain MRI on day X+2 revealed multiple hyperintense lesions scattered in the bilateral cerebral hemispheres on diffusion-weighted imaging (DWI), leading to a diagnosis of cerebral fat embolism (CFE). Despite treatment with methylprednisolone and protirelin, his condition did not improve. Nonconvulsive status epilepticus was treated with levetiracetam; however, consciousness remained impaired. Serial brain MRI revealed progressive confluence of white matter lesions. Hyperintense areas on DWI in the cerebral white matter persisted into the subacute and chronic phases. Together with previous reports, this case highlights the potential association between persistence of DWI hyperintensity approximately one month after onset and poor neurological outcomes in patients with CFE.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"78-84"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145985689","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 neurosyphilis presenting with acute right hemiparesis improved by adjunctive steroid therapy].","authors":"Mai Iwahara, Nanako Ono, Yoji Kasajima, Yasuhisa Akaiwa, Toshiki Nakamura, Tomoyuki Miyamoto","doi":"10.5692/clinicalneurol.cn-002177","DOIUrl":"10.5692/clinicalneurol.cn-002177","url":null,"abstract":"<p><p>A 57-year-old man was rushed to our hospital with acute right hemiplegia. Head MRI revealed high signals in the left motor cortex that did not correspond to vascular control, mainly in the cortex, on DWI. However, serum and cerebrospinal fluid were positive for syphilis antibodies, cerebrospinal fluid protein was elevated, and there was no atrial fibrillation. Head MRA and MRV were normal, so the patient was diagnosed with neurosyphilis and antisyphilis therapy was administered. However, the right hemiplegia did not improve much, so an additional steroid was administered, which resulted in a marked improvement in clinical symptoms. It is thought that the steroid contributed to the improvement of symptoms in this case due to vasculitis caused by Treponema pallidum, an excessive immunological response to the destruction of the bacteria, and cytokine release due to inflammation. Additional administration of steroids may be considered in addition to antisyphilis therapy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"85-89"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145985783","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":"Addendum to the 2023 clinical practice guidelines for amyotrophic lateral sclerosis in Japan: approval and integration of novel disease-modifying therapies.","authors":"Hitoshi Warita, Makoto Urushitani, Naoki Atsuta, Yuishin Izumi, Osamu Kano, Toshio Shimizu, Yuki Nakayama, Yugo Narita, Hiroyuki Nodera, Takuji Fujita, Koichi Mizoguchi, Mitsuya Morita, Masashi Aoki","doi":"10.5692/clinicalneurol.cn-002198","DOIUrl":"10.5692/clinicalneurol.cn-002198","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) is an intractable motor neuron disease characterized by progressive degeneration of motor neurons with varying degrees of frontotemporal lobe dysfunction. This English summary of the addendum to the Japanese clinical practice guidelines for ALS outlines major recent advances in pharmacological therapy in Japan. Following the development of the 2023 guidelines, three additional medications-oral edaravone, high-dose intramuscular mecobalamin, and tofersen-have been introduced. Oral edaravone, with its ease of administration, demonstrates pharmacokinetics comparable to the intravenous formulation. High-dose mecobalamin reduces functional decline when initiated early in the disease course. Tofersen, an antisense oligonucleotide, is the first gene-targeted therapy approved in Japan for patients with copper/zinc superoxide dismutase gene-related ALS, highlighting the importance of genetic testing and counseling in all ALS cases. This addendum provides updated expert consensus recommendations for the use, dosing, and monitoring of these therapies, while emphasizing the need for thorough communication about the ethical and psychological dimensions of genetic testing. It also addresses practical considerations for combination therapy, noting that up to three or four anti-ALS agents are now available in Japan. The long-term safety and efficacy of these therapies, as well as their potential synergistic or additive effects, remain to be clarified through real-world data and prospective registries. The objectives of this addendum are twofold: to present these advances and recommendations in English to foster international collaboration, and to inform the global ALS community about the latest therapeutic strategies in Japan. In addition, ongoing efforts to harmonize clinical evaluation standards and promote international clinical trials are highlighted, with the goal of improving patient outcomes and advancing ALS research worldwide.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"67-73"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019948","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":"[An autopsy case of glioblastoma with diffuse leptomeningeal spread that was difficult to diagnose before death].","authors":"Fujio Umehara, Mari Kirishima","doi":"10.5692/clinicalneurol.cn-002187","DOIUrl":"10.5692/clinicalneurol.cn-002187","url":null,"abstract":"<p><p>The patient was a male in his 50s. He had been aware of headache since the end of April, X year, and was admitted to the Neurosurgery Department of X Hospital in early May; however, his headache and posterior neck pain worsened. He was discharged from the previous hospital and admitted to this hospital in late May. His consciousness was clear, and there were no abnormalities in his cranial nerves or limb motor systems. Tendon reflexes were normal and Babinski's sign was negative. He complained of posterior neck pain and remained in bed all day with his neck flexed backward. Head MRI showed a high fluid-attenuated inversion recovery (FLAIR) signal, high diffusion-weighted imaging (DWI) signal, and low T₂* signal around the inferior horn of left lateral ventricle, and contrast-enhanced MRI showed a diffuse contrast effect on the cerebral surface, brainstem, and spinal cord soft membrane. Cerebrospinal fluid (CSF) analysis showed 98 cells/μl (66% mononuclear cells, 34% polymorphonuclear cells), protein level of 140 ‍mg/dl, and glucose level of 32 ‍mg/dl (simultaneous blood glucose 160 ‍mg/dl); cytology was negative. Malignant lymphoma, fungal/tuberculous meningitis, meningeal carcinomatosis, and granulomatous disease were suspected; however, no abnormalities were observed. In mid-June, the patient's condition suddenly deteriorated, and he died. Autopsy revealed an epithelioid glioblastoma (isocitrate dehydrogenase [IDH]-wild-type, World Health Organization [WHO] grade IV) near the left subventricular angle, with diffuse infiltration of the glioblastoma on the cerebral surface, brainstem, and spinal cord pia mater. The patient was diagnosed with glioblastoma with diffuse leptomeningeal spread.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"90-97"},"PeriodicalIF":0.0,"publicationDate":"2026-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019983","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}