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[Beta-propeller protein-associated neurodegeneration (BPAN) associated with heper-CKemia-case report]. [β -螺旋桨蛋白相关神经退行性变（BPAN）与高血压-肾病病例报告]。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-22 DOI: 10.5692/clinicalneurol.cn-002147
Tomoko Sugiyama, Mizuki Matsumoto, Shinya Okuda, Marina Mizobe, Kazuhiro Muramatsu, Masaru Matsui
{"title":"[Beta-propeller protein-associated neurodegeneration (BPAN) associated with heper-CKemia-case report].","authors":"Tomoko Sugiyama, Mizuki Matsumoto, Shinya Okuda, Marina Mizobe, Kazuhiro Muramatsu, Masaru Matsui","doi":"10.5692/clinicalneurol.cn-002147","DOIUrl":"10.5692/clinicalneurol.cn-002147","url":null,"abstract":"<p><p>Beta-propeller protein-associated neurodegeneration (BPAN) is a neurodegeneration with brain iron accumulation (NBIA) disorder caused by autophagy abnormalities. Clinical features of BPAN include global developmental delay in early childhood, followed by progression of cognitive dysfunction and parkinsonism in adulthood. A 32-year-old woman diagnosed with BPAN and confirmed by genetic analysis showed motor symptoms that rapidly progressed after the age of 30 years. Baclofen was administered for spasticity, though a high fever and elevated serum CK level were observed, and the symptoms persisted for several months even after stopping the drug. It was suspected that rhabdomyolysis have been due to muscle tissue fragility associated with autophagy impairment in BPAN, in combination with increased muscle tone.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"389-394"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148017876","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
[Anti-leucine-rich glioma-inactivated 1 antibody-positive encephalitis with a poor response to corticosteroids 2 months after onset of impaired memory: a case report]. [抗富亮氨酸胶质瘤失活抗体阳性脑炎伴皮质类固醇不良反应，发病后2个月记忆受损：1例报告]。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-15 DOI: 10.5692/clinicalneurol.cn-002223
Yukako Araga, Yoshitsugu Nakamura, Chizuko Sugita, Makoto Hara, Hideto Nakajima, Shigeki Arawaka
{"title":"[Anti-leucine-rich glioma-inactivated 1 antibody-positive encephalitis with a poor response to corticosteroids 2 months after onset of impaired memory: a case report].","authors":"Yukako Araga, Yoshitsugu Nakamura, Chizuko Sugita, Makoto Hara, Hideto Nakajima, Shigeki Arawaka","doi":"10.5692/clinicalneurol.cn-002223","DOIUrl":"10.5692/clinicalneurol.cn-002223","url":null,"abstract":"<p><p>The patient was a 30-year-old man who was admitted to our department 2 months after the onset of anterograde amnesia. Cognitive function tests indicated memory impairment only. MRI FLAIR images of the head showed a high signal in the bilateral medial temporal lobes. Corticosteroid therapy did not improve his symptoms, and he subsequently developed status epilepticus. Concurrently, his cerebrospinal fluid was positive for anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. He was diagnosed with anti-LGI1 antibody-positive encephalitis. Plasma exchange therapy and intravenous immunoglobulin therapy improved the status epilepticus, but memory impairment and bilateral medial temporal lobe atrophy remained. This case was characterized by memory impairment that only lasted for 2 months after onset and a poor response to corticosteroid therapy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"409-415"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147964760","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
[Hemiplegic migraine: from historical perspectives to current management]. [偏瘫偏头痛：从历史角度到目前的管理]。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-22 DOI: 10.5692/clinicalneurol.cn-002244
Mamoru Shibata, Kazumasa Saigoh, Daisuke Danno, Eiji Kitamura, Tsubasa Takizawa, Yuya Kano
{"title":"[Hemiplegic migraine: from historical perspectives to current management].","authors":"Mamoru Shibata, Kazumasa Saigoh, Daisuke Danno, Eiji Kitamura, Tsubasa Takizawa, Yuya Kano","doi":"10.5692/clinicalneurol.cn-002244","DOIUrl":"10.5692/clinicalneurol.cn-002244","url":null,"abstract":"<p><p>Hemiplegic migraine is a rare subtype of migraine characterized by reversible hemiplegia/hemiparesis as an aura symptom. Visual, sensory, and/or speech symptoms typically accompany the motor aura, followed by throbbing headache. Because motor symptoms may last for up to several weeks, this unique clinical entity can markedly impair quality of life. Familial hemiplegic migraine is caused by autosomal dominant mutations in the CACNA1A, ATP1A2, SCN1A, and PRRT2 genes. These mutations are thought to induce neuronal hyperexcitability with resultant enhanced synaptic transmission and increased susceptibility to cortical spreading depolarization/depression. Therapeutic options remain limited, owing to the contraindication of triptans for acute treatment and the lack of established preventive therapies. However, recent evidence supports the potential efficacy of calcitonin gene-related peptide-targeting monoclonal antibodies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"375-383"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148017824","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
Before a spinal cord lesion in an m.3243A>G carrier is attributed to mitochondrial disease, all differential diagnoses must be excluded. 在将m.3243A b> G携带者的脊髓病变归因于线粒体疾病之前，必须排除所有鉴别诊断。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-16 DOI: 10.5692/clinicalneurol.cn-002190
Josef Finsterer
{"title":"Before a spinal cord lesion in an m.3243A>G carrier is attributed to mitochondrial disease, all differential diagnoses must be excluded.","authors":"Josef Finsterer","doi":"10.5692/clinicalneurol.cn-002190","DOIUrl":"10.5692/clinicalneurol.cn-002190","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"420-421"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147964934","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 IgG4-related hypertrophic pachymeningitis and leptomeningitis presenting with cognitive impairment]. [1例与igg4相关的肥厚性厚性脑膜炎和轻脑膜炎表现为认知障碍]。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-22 DOI: 10.5692/clinicalneurol.cn-002179
Ryuta Maekawa, Ryutaro Matsuoka, Yasuhiro Maeda, Takeshi Fujimoto
{"title":"[A case of IgG4-related hypertrophic pachymeningitis and leptomeningitis presenting with cognitive impairment].","authors":"Ryuta Maekawa, Ryutaro Matsuoka, Yasuhiro Maeda, Takeshi Fujimoto","doi":"10.5692/clinicalneurol.cn-002179","DOIUrl":"10.5692/clinicalneurol.cn-002179","url":null,"abstract":"<p><p>A 68-year-old female was admitted to our hospital because of a right-sided headache since one year, an inability to perform housework, and a decrease in speech since March of that year. Upon admission, higher brain dysfunctions, including attention disorder, aphasia, and apraxia, were observed. Contrast-enhanced MRI revealed dural thickening in the bilateral frontal to parietal lobes and FLAIR imaging revealed high signal intensities in the bilateral frontal brain sulcus. Blood tests revealed a high IgG4 level (273 mg/dl). Dural biopsy revealed infiltration of IgG4-positive plasma cells into the tissue, and IgG4-related hypertrophic pachymeningitis was diagnosed. Administration of prednisolone resulted in resolution of the dural thickening, an absence of FLAIR imaging high signal intensities of the cerebral sulcus, and a considerable improvement of the higher brain dysfunctions. In patients with headache and higher brain dysfunctions, hypertrophic pachymeningitis should be considered in the differential diagnosis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"395-400"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148017848","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脊髓受累一例。
Clinical Neurology Pub Date : 2026-06-20 Epub Date: 2026-05-16 DOI: 10.5692/clinicalneurol.cn-002220
Ken-Ichi Shibata, Tatsuya Mukai, Hideaki Nakagaki, Sukehisa Nagano
{"title":"A case of a spinal cord involvement in an adult mitochondrial disease with an m.3243A>G.","authors":"Ken-Ichi Shibata, Tatsuya Mukai, Hideaki Nakagaki, Sukehisa Nagano","doi":"10.5692/clinicalneurol.cn-002220","DOIUrl":"10.5692/clinicalneurol.cn-002220","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"422-423"},"PeriodicalIF":0.0,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147964809","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 slowly progressive cauda equina syndrome caused by invasive lobular carcinoma of the left breast with leptomeningeal carcinomatosis]. 【左乳浸润性小叶癌合并轻脑膜癌病变致慢进行性马尾综合征1例】。
Clinical Neurology Pub Date : 2026-05-26 Epub Date: 2026-04-11 DOI: 10.5692/clinicalneurol.cn-002215
Yuri Kunisada, Tomoko Tanaka, Shusuke Koto, Takaaki Nakanishi, Mayuko Miki, Norio Chihara
{"title":"[A case of slowly progressive cauda equina syndrome caused by invasive lobular carcinoma of the left breast with leptomeningeal carcinomatosis].","authors":"Yuri Kunisada, Tomoko Tanaka, Shusuke Koto, Takaaki Nakanishi, Mayuko Miki, Norio Chihara","doi":"10.5692/clinicalneurol.cn-002215","DOIUrl":"10.5692/clinicalneurol.cn-002215","url":null,"abstract":"<p><p>Herein, we present the case of a 67-year-old woman who presented with a 14-month history of progressive lower limb paresthesia and muscle weakness. Bilateral sensorineural hearing loss also developed during the disease course. Six months prior to admission, cerebrospinal fluid cytology revealed findings suspect of malignancy. Although tumor screening was performed, the cause could not be identified. Due to symptom progression, the patient was re-admitted after which further examination revealed invasive lobular carcinoma of the left breast as the primary tumor, with further metastasis to the large intestine also confirmed. Immunostaining of the cerebrospinal fluid revealed cells with positive findings for estrogen receptor (ER) and AE1/3, leading to a definitive diagnosis of meningeal dissemination. This was a rare case of meningeal dissemination of breast cancer, initially presenting with slowly progressive cauda equina syndrome. This case highlights the importance of repeated tumor screening for neurological symptoms of unknown primary origin.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"309-313"},"PeriodicalIF":0.0,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147677502","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 young adult with internal carotid artery occlusion during finasteride therapy: pathological findings from a thrombus retrieved by mechanical thrombectomy]. [非那雄胺治疗期间颈内动脉闭塞的年轻人：机械取栓术取出血栓的病理结果]。
Clinical Neurology Pub Date : 2026-05-26 Epub Date: 2026-04-21 DOI: 10.5692/clinicalneurol.cn-002214
Noriaki Tashiro, Masahiro Yasaka, Takashi Fujii, Kosuke Takigawa, Yoshihiro Fukui, Shuta Maehara
{"title":"[A young adult with internal carotid artery occlusion during finasteride therapy: pathological findings from a thrombus retrieved by mechanical thrombectomy].","authors":"Noriaki Tashiro, Masahiro Yasaka, Takashi Fujii, Kosuke Takigawa, Yoshihiro Fukui, Shuta Maehara","doi":"10.5692/clinicalneurol.cn-002214","DOIUrl":"10.5692/clinicalneurol.cn-002214","url":null,"abstract":"<p><p>Androgenetic alopecia (AGA) is commonly treated with finasteride and minoxidil, which are both recommended as grade A therapies in the Japanese Dermatological Association guidelines. However, their association with thromboembolic events and ischemic stroke remains unclear. We report a 40-year-old man without apparent risk factors for stroke who developed acute ischemic stroke due to left internal carotid artery occlusion while taking finasteride (1 mg/day) and minoxidil (10 mg/day). Mechanical thrombectomy retrieved a dark red thrombus primarily composed of red blood cells and fibrin. This case represents a rare report of internal carotid artery occlusion with pathologically confirmed thrombus in a patient receiving these medications. Finasteride may promote hypercoagulability via androgen suppression-induced estrogen predominance, and minoxidil may contribute through blood pressure-lowering effects. Intravenous alteplase and thrombectomy were effective treatments, and discontinuation of the medications is recommended for secondary prevention.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"304-308"},"PeriodicalIF":0.0,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147784364","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
[Dystrophia myotonica type 1 associated with diffuse astrocytoma showing gliomatosis cerebri-like imaging finding: a case report]. [1型肌强直营养不良伴弥漫性星形细胞瘤，影像学表现为脑样胶质瘤：1例报告]。
Clinical Neurology Pub Date : 2026-05-26 Epub Date: 2026-04-21 DOI: 10.5692/clinicalneurol.cn-002205
Shumpei Matsumoto, Yuichiro Isozawa, Tetsuya Nagatani, Yasushi Iwasaki, Keizo Yasui
{"title":"[Dystrophia myotonica type 1 associated with diffuse astrocytoma showing gliomatosis cerebri-like imaging finding: a case report].","authors":"Shumpei Matsumoto, Yuichiro Isozawa, Tetsuya Nagatani, Yasushi Iwasaki, Keizo Yasui","doi":"10.5692/clinicalneurol.cn-002205","DOIUrl":"10.5692/clinicalneurol.cn-002205","url":null,"abstract":"<p><p>The patient was a 43-year-old woman. She had been aware of progressive muscle weakness since around the age of 30. Two months ago, she began to have difficulty making sense of conversation. She was transported to our hospital after choking, and upon arrival, she was found to have generalized muscle weakness and myotonia, along with disorientation. A brain MRI revealed widespread high-intensity lesions in the cerebral white matter on FLAIR images, findings similar to gliomatosis cerebri (GC). Genetical analysis revealed a diagnosis of dystrophia myotonica type 1 (DM1). A brain biopsy revealed a diffuse astrocytoma. There have been no previous reports of DM1 associated with diffuse astrocytoma and imaging findings similar to GC, and we report this patient together with a literature review.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"299-303"},"PeriodicalIF":0.0,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147784407","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
Nivolumab-related encephalitis with extensive cortical-subcortical involvement. 伴有广泛皮层-皮层下受累的尼伏单抗相关性脑炎。
Clinical Neurology Pub Date : 2026-05-26 Epub Date: 2026-04-21 DOI: 10.5692/clinicalneurol.cn-002192
Kei Matsuyama, Keisuke Shima, Takeshi Tsuda, Hitoshi Abo, Hirokazu Taniguchi
{"title":"Nivolumab-related encephalitis with extensive cortical-subcortical involvement.","authors":"Kei Matsuyama, Keisuke Shima, Takeshi Tsuda, Hitoshi Abo, Hirokazu Taniguchi","doi":"10.5692/clinicalneurol.cn-002192","DOIUrl":"10.5692/clinicalneurol.cn-002192","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"334-335"},"PeriodicalIF":0.0,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147784403","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
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