Clinical Neurology最新文献_第2页

[Anti-NXP2 antibody-positive dermatomyositis showing marked subcutaneous edema predominant in the upper limbs]. [抗nxp2抗体阳性皮肌炎表现为上肢明显的皮下水肿]。

Clinical Neurology Pub Date : 2025-09-25 Epub Date: 2025-08-22 DOI: 10.5692/clinicalneurol.cn-002118

Nozomu Abe, Satoko Uruha, Akinori Uruha, Shinsuke Tobisawa, Kazushi Takahashi

引用次数: 0

[An autopsy case of primary progressive multiple sclerosis with minimal acute inflammation and remyelination over an 11-year course]. [11年的尸检过程中，伴有轻微急性炎症和髓鞘再生的原发性进行性多发性硬化症病例]。

Clinical Neurology Pub Date : 2025-09-25 Epub Date: 2025-08-28 DOI: 10.5692/clinicalneurol.cn-002092

Kohei Asano, Kuniyuki Iwata-Endo, Amane Araki, Saori Morozumi, Yasushi Iwasaki, Keizo Yasui

引用次数: 0

[Questionnaire survey about barriers to telemedicine for patients with intractable neurological disorders]. [难治性神经系统疾病患者远程医疗障碍问卷调查]。

Clinical Neurology Pub Date : 2025-09-25 Epub Date: 2025-08-28 DOI: 10.5692/clinicalneurol.cn-002106

Yuki Tajiri, Yo Shibata, Takahiro Shimizu, Hiroshi Takigawa, Yoshinori Ishida, Yoshikazu Ugawa, Yoshio Tsuboi, Ritsuko Hanajima

{"title":"[Questionnaire survey about barriers to telemedicine for patients with intractable neurological disorders].","authors":"Yuki Tajiri, Yo Shibata, Takahiro Shimizu, Hiroshi Takigawa, Yoshinori Ishida, Yoshikazu Ugawa, Yoshio Tsuboi, Ritsuko Hanajima","doi":"10.5692/clinicalneurol.cn-002106","DOIUrl":"10.5692/clinicalneurol.cn-002106","url":null,"abstract":"Telemedicine is not widely used in patients with intractable neurological disorders including neurodegenerative diseases even though its application was covered by national insurance in Japan. To clarify what patient factors should be overcome for telemedicine application, we surveyed outpatients with intractable neurological disorders using questionnaires in Tottori University Hospital, Tekeda General Hospital and Fukuoka University Hospitals. The survey showed that more than half of the telemedicine-beginners did not want to use telemedicine in the former two hospitals located in provincial cities (0.2 to 0.3 million population), but more than half would like to use it in the large hospital sited at one of the regional core cites (1.5 million population). Binomial logistic analysis of the beginners revealed that predictors for patients to choose \"I do not want to use telemedicine\" were \"no experience using internet at home\" and \"need of a helper to use internet equipment for telemedicine at home\". The odds ratios of the other factors such as the concerns about the cost or quality of outpatient medical practices were low. On the other hand, most of already telemedicine users wanted to continue telemedicine. The present study revealed that the quality of internet connection environment is the most critical factor for patients to use telemedicine. It must be requisite for wide use of telemedicine to establish a good internet connection environment at areas/home and to supply good equipment to the patients for the connection.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"637-646"},"PeriodicalIF":0.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144973064","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}

引用次数: 0

[Pisa syndrome with laterocollis associated with bilateral chronic subdural hematomas: a case report with reference to peripheral vestibular hypofunction]. [比萨综合征伴侧结肠合并双侧慢性硬膜下血肿：前庭周围功能减退1例]。

Clinical Neurology Pub Date : 2025-09-25 Epub Date: 2025-08-22 DOI: 10.5692/clinicalneurol.cn-002130

Motomi Arai

{"title":"[Pisa syndrome with laterocollis associated with bilateral chronic subdural hematomas: a case report with reference to peripheral vestibular hypofunction].","authors":"Motomi Arai","doi":"10.5692/clinicalneurol.cn-002130","DOIUrl":"10.5692/clinicalneurol.cn-002130","url":null,"abstract":"An 88-year-old woman with a history of two prior episodes of vertigo developed a mild leftward head tilt approximately four months after initiating pregabalin for severe tingling in the distal extremities. Two months later (day 1), she sustained a head injury without loss of consciousness. On day 22, she acutely developed leftward lateral flexion of the head while standing. Her gait progressively deteriorated, and she presented to our clinic on day 38. Neurological examination revealed mild disturbance of consciousness and marked leftward tilt of the head and trunk. Cranial MRI demonstrated bilateral chronic subdural hematomas without intra-axial lesions, including in the brainstem or cerebellum. Following surgical evacuation, the abnormal posture of the head and trunk resolved promptly. A horizontal, direction-fixed, right-beating nystagmus was observed, suggesting left-sided vestibular hypofunction. Subtle postural imbalance due to vestibular hypofunction and adverse effects of pregabalin may have been decompensated by the subdural hematomas, resulting in the pronounced cervical and truncal tilt.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"676-678"},"PeriodicalIF":0.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144973107","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}

引用次数: 0

[AQP4 positive neuromyelitis optica spectrum disorder: an atypical pathology with nerve root enlargement and peripheral nervous system damage in demyelination]. [AQP4阳性视神经脊髓炎频谱障碍：脱髓鞘中神经根扩大和周围神经系统损害的非典型病理]。

Clinical Neurology Pub Date : 2025-09-25 Epub Date: 2025-08-22 DOI: 10.5692/clinicalneurol.cn-002077

Eiji Ogawa, Mizuki Otomo, Kenichi Tsukita, Aki Yasaka, Genya Watanabe, Yasushi Suzuki

{"title":"[AQP4 positive neuromyelitis optica spectrum disorder: an atypical pathology with nerve root enlargement and peripheral nervous system damage in demyelination].","authors":"Eiji Ogawa, Mizuki Otomo, Kenichi Tsukita, Aki Yasaka, Genya Watanabe, Yasushi Suzuki","doi":"10.5692/clinicalneurol.cn-002077","DOIUrl":"10.5692/clinicalneurol.cn-002077","url":null,"abstract":"A 72-year-old woman with limb weakness was admitted to our hospital. Her symptoms began just one month prior to presentation and showed gradual progression, leading to difficulties in physical movement. She had undergone breast cancer surgery at the age of 70 years. Neoadjuvant chemotherapy with epirubicin, cyclophosphamide, and docetaxel was administered, and the patient subsequently underwent a simple mastectomy and axillary lymphadenectomy. Neurological examination revealed bilateral relative afferent pupillary defects, severe muscle weakness in the limbs, hyporeflexia of the lower extremities, sensory disturbances below Th1 level, dysuria, and constipation. MRI revealed a left internal capsule lesion, extensive longitudinal transverse myelitis at C4-Th10 level, nerve root enlargement in the extraspinal canal, and high bilateral cervical/lumbar cord intensity. Nerve conduction studies revealed bilateral demyelinating polyneuropathy of the median nerves. Positive anti-AQP4 antibodies in both serum and cerebrospinal fluid ‍led to the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). The patient's symptoms improved after immunosuppressive treatment. Here, we describe the case of a patient with AQP4 autoantibody-positive NMOSD who demonstrated nerve root enlargement in the extraspinal canal and peripheral nervous system damage during demyelination.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"647-654"},"PeriodicalIF":0.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144973121","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}

引用次数: 0

[A case of a spinal cord involvement in an adult mitochondrial disease with an m.3243A>G]. [成人线粒体病伴m.3243A>G累及脊髓一例]。

Clinical Neurology Pub Date : 2025-08-28 Epub Date: 2025-07-24 DOI: 10.5692/clinicalneurol.cn-002071

Ken-Ichi Shibata, Tatsuya Mukai, Hideaki Nakagaki, Sukehisa Nagano

引用次数: 0

[Hypertrophy of the lumbosacral nerve roots in Noonan syndrome with multiple lentigines: a case report]. 【努南综合征伴多椎体的腰骶神经根肥大1例】。

Clinical Neurology Pub Date : 2025-08-28 Epub Date: 2025-07-24 DOI: 10.5692/clinicalneurol.cn-002094

Yukako Araga, Yoshitsugu Nakamura, Kensuke Kakiuchi, Takafumi Hosokawa, Shimon Ishida, Shigeki Arawaka

{"title":"[Hypertrophy of the lumbosacral nerve roots in Noonan syndrome with multiple lentigines: a case report].","authors":"Yukako Araga, Yoshitsugu Nakamura, Kensuke Kakiuchi, Takafumi Hosokawa, Shimon Ishida, Shigeki Arawaka","doi":"10.5692/clinicalneurol.cn-002094","DOIUrl":"10.5692/clinicalneurol.cn-002094","url":null,"abstract":"The patient was a 61-year-old man in whom sensorineural hearing loss were noted after birth and the presence of multiple cutaneous millet-sized lentigines were noted after about 6-year-old. He had pain in his bilateral lower extremities; 1 month later, he visited our hospital. He had no family history of neurological or cutaneous symptoms. In nerve conduction studies, the F-wave frequencies were reduced in the bilateral tibial nerves. In lumbar spine magnetic resonance imaging, the bilateral lumbosacral nerve roots showed hypertrophy. A genetic analysis revealed that he had a heterozygous single-base non-synonymous substitution in the PTPN11 gene (c.1403C>T, p.Thr468Met). This substitution is known pathogenic variant. We diagnosed the patient with Noonan syndrome with multiple lentigines. This syndrome is a RASopathy that is caused by variants in genes encoding the SHP-2 protein, which is a component of the RAS/mitogen-activated protein kinase (MAPK) signaling pathway. RASopathies should be included as differential diagnoses for spinal nerve root enlargement.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"595-600"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709303","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}

引用次数: 0

[An autopsy case of non-drug related progressive multifocal leukoencephalopathy in a background of rheumatoid arthritis]. [一例以类风湿关节炎为背景的非药物相关性进行性多灶性脑白质病的尸检]。

Clinical Neurology Pub Date : 2025-08-28 Epub Date: 2025-07-24 DOI: 10.5692/clinicalneurol.cn-002080

Yoshie Kato, Asuka Araki, Yoshiharu Miura, Kazuo Nakamichi, Kenta Takahashi, Atsushi Nagai

{"title":"[An autopsy case of non-drug related progressive multifocal leukoencephalopathy in a background of rheumatoid arthritis].","authors":"Yoshie Kato, Asuka Araki, Yoshiharu Miura, Kazuo Nakamichi, Kenta Takahashi, Atsushi Nagai","doi":"10.5692/clinicalneurol.cn-002080","DOIUrl":"10.5692/clinicalneurol.cn-002080","url":null,"abstract":"A 56-year-old male subject was presented with a two-month history of dysarthria and gait disturbance. T2-weighted MRI revealed high signal intensity in the right cerebellar hemisphere, pons, and bilateral middle cerebellar peduncles. Suspecting brain-stem encephalitis, he was treated with steroid pulse therapy, which was ineffective. Subsequently, a qualitative PCR test was performed, confirming the diagnosis of progressive multifocal leukoencephalopathy (PML) with a high JC virus (JCV) load (273,857 copies/ml) in the cerebrospinal fluid (CSF). The JCV strain in the CSF was a prototype with a deletion in the regulatory region of its genomic DNA. Long-term untreated rheumatoid arthritis (RA) was identified as an underlying cause. Other potential immunodeficiency-related diseases, including idiopathic CD4 lymphocytopenia, malignant RA, systemic lupus erythematosus, chronic eosinophilic leukemia, malignant lymphoma, and congenital immunodeficiency, were ruled out based on various laboratory tests. Despite treatment with a combination of mefloquine and mirtazapine, the patient died on the 102nd day due to disease progression. RA with non-drug related immune abnormalities should be considered a potential underlying cause of PML.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"582-587"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709301","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}

引用次数: 0

[A case of recurrent cerebellitis leading to the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)]. [一例复发性小脑炎导致线粒体脑肌病、乳酸酸中毒和卒中样发作（MELAS）的诊断]。

Clinical Neurology Pub Date : 2025-08-28 Epub Date: 2025-07-24 DOI: 10.5692/clinicalneurol.cn-002117

Yuko Ito, Chikako Ochi, Yuki Yamanishi, Hiroshi Takashima, Akihiro Hashiguchi, Masahiro Nagai

{"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-002117","DOIUrl":"10.5692/clinicalneurol.cn-002117","url":null,"abstract":"The patient was a 41-year-old man with a history of diabetes mellitus since the age of 22 years and a family history of diabetes in his mother. He had repeated headaches, cerebellar symptoms, and bilateral cerebellar lesions on brain MRI (DWI, T2 FLAIR hyperintensities). He was previously diagnosed with cerebellitis and treated with steroids at another hospital. Upon his third relapse, he was referred to our department. Clinical findings included short stature, diabetes, elevated CSF lactate and pyruvate levels, and increased lactate levels on aerobic exercise testing. Muscle biopsy revealed mitochondrial pathological abnormalities, and genetic testing of muscle tissue identified the mitochondrial DNA m.3243 mutation. These findings led to the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). Stroke-like lesions in MELAS can involve not only the cerebrum but also the cerebellum. When cortical-predominant lesions with associated vascular dilatation are observed, MELAS should be considered in the differential diagnosis.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"601-606"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709298","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}

引用次数: 0

[Received the Japan Neurological Society Award]. [获得日本神经学会奖]。

Clinical Neurology Pub Date : 2025-08-28 Epub Date: 2025-07-24 DOI: 10.5692/clinicalneurol.cn-002139

Takashi Kanda

引用次数: 0