Clinical Neurology最新文献

{"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":"Cortical hyperperfusion and subsequent atrophy in neuronal intranuclear inclusion disease.","authors":"Tetsuro Shirakawa, Ryo Sasaki, Yoshiaki Takahashi, Jun Sone, Nobutoshi Morimoto","doi":"10.5692/clinicalneurol.cn-002254","DOIUrl":"10.5692/clinicalneurol.cn-002254","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"568-569"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413017","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":"[Utility of acute-phase cerebral blood flow single photon emission computed tomography (SPECT) for evaluating the pathophysiology of Bickerstaff brainstem encephalitis with decorticate posturing].","authors":"Tamon Koga, Ken-Ichi Irie, Shinichiro Mori, Masahiko Koga, Tatsuya Imai, Takahisa Tateishi","doi":"10.5692/clinicalneurol.cn-002265","DOIUrl":"10.5692/clinicalneurol.cn-002265","url":null,"abstract":"<p><p>Bickerstaff's brainstem encephalitis (BBE) is an autoimmune disorder typically localized to the brainstem. While cortical involvement is rare, severe cases presenting with decorticate posturing and profound consciousness disturbance can mask typical clinical features, making early diagnosis challenging. A 22-year-old woman developed progressive numbness in her limbs and lips, visual impairment, and impaired consciousness 13 days after an episode of gastroenteritis. Upon admission, she exhibited a Japan Coma Scale score of III-200, decorticate posturing, absent brainstem reflexes, and hyperreflexia. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein levels. Although contrast-enhanced MRI showed only faint enhancement along the leptomeninges of the cerebral cortex and midbrain, acute-phase ¹²³I-IMP cerebral blood flow (CBF) single photon emission computed tomography (SPECT) demonstrated diffuse cortical hypoperfusion and paradoxical hyperperfusion in the bilateral basal ganglia. Furthermore, multiple autoantibodies associated with autoimmune encephalitis and demyelinating diseases-including anti-NMDAR, LGI1, CASPR2, AMPAR, GABABR, DPPX, GlyR, mGluR5, MOG, and AQP4 antibodies-were all negative in both serum and cerebrospinal fluid. Following aggressive immunotherapy, her consciousness improved rapidly. It was only during this recovery phase that the classic BBE triad of total ophthalmoplegia and ataxia became apparent, which had been masked by the initial severe consciousness disturbance. The diagnosis of BBE was retrospectively confirmed based on this clinical evolution and the high titers of serum anti-GQ1b and anti-GT1a IgG antibodies. Follow-up SPECT confirmed the normalization of CBF, correlating with her clinical recovery. This case demonstrates that cerebral perfusion SPECT is capable of detecting reversible dysfunction within the cortex and basal ganglia in BBE. It highlights the importance of considering BBE even in atypical cases with predominant cortical symptoms, where the diagnosis may only become clear through clinical evolution and ancillary functional imaging.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"559-563"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392097","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":"[Transient myoclonic state with asterixis related to COVID-19].","authors":"Koki Kikuchi, Kenji Sakai","doi":"10.5692/clinicalneurol.cn-002235","DOIUrl":"10.5692/clinicalneurol.cn-002235","url":null,"abstract":"<p><p>A 64-year-old Japanese man presented with flu-like symptoms and received a diagnosis of COVID-19 (day 1). The patient developed sudden-onset involuntary movements in his trunk, left upper limb, and face (day 7). On examination (day 8), myoclonus was observed on the bilateral upper limbs and the trunk. Asterixis was also noted on the bilateral hands at the dorsiflex position of the wrists. Other neurological manifestations were unremarkable. After initiation of oral clonazepam (1.5 mg/day) administration, the involuntary movements vanished within several hours. No recurrence of the neurological symptoms, including any involuntary movements, was observed afterwards. Based on the clinical course, the patient was diagnosed with transient myoclonic state with asterixis (TMA) associated with COVID-19. Respiratory infections could cause TMA; however, there have been no clinical reports of TMA caused by COVID-19. Hypercytokinemia followed by COVID-19 and immune reaction against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can be related to transient dysfunction of the central nervous system, such as motor cortex, brain stem, and hypothalamus, which could be associated with development of TMA.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"535-539"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392053","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":"[Steroid responsive rapidly progressive HTLV-1-associated myelopathy with HTLV-1-associated bronchioloalveolar disease: a case report].","authors":"Ririna Demizu, Yoshihisa Otsuka, Ryogo Tamada, Yu Mizutani","doi":"10.5692/clinicalneurol.cn-002251","DOIUrl":"10.5692/clinicalneurol.cn-002251","url":null,"abstract":"<p><p>An 84-year-old woman presented with a two months history of progressive bilateral lower limb weakness and bladder bowel disfunction. Neurological examination revealed spastic paraplegia and anti HTLV-1 antibodies were positive in the serum and cerebrospinal fluid. Due to the rapid progression along with the elevation of neopterin and CXCL-10 in the cerebrospinal fluid, the diagnosis of rapidly progressive HTLV-1 associated myelopathy (HAM) was made. Chest X-ray and CT coincidentally revealed diffuse micronodular opacities, nodular in various sizes, and interlobular septal thickening. HTLV-1 associated bronchioalveolar disorder was the most likely diagnosis. Steroid therapy was effective against both spastic paraplegia and pulmonary lesions. Although the pulmonary lesions were identified incidentally in our case, screening for pulmonary lesions is important in patients with spastic paraplegia as it may lead to early diagnosis of HAM.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"564-567"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412970","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 management of headache comorbid with functional neurological disorder].","authors":"Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-002260","DOIUrl":"10.5692/clinicalneurol.cn-002260","url":null,"abstract":"<p><p>Headache, particularly migraine, is highly prevalent in patients with functional neurological disorder (FND) but is often underrecognized in clinical practice. This review provides an overview of the existing literature on the association between FND and headache and demonstrates that comorbid migraine is associated with greater disease severity and increased clinical complexity. FND and migraine may share, at least in part, common pathophysiological mechanisms. In clinical practice, careful attention should be paid to avoiding both underdiagnosis and overdiagnosis, and an integrated therapeutic approach is warranted.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"523-528"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392084","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 female dystrophinopathy with presumed childhood onset, presenting with asymmetric muscle weakness and delayed diagnosis in old age].","authors":"Rie Tohge, Yasumasa Hashimoto, Ichizo Nishino, Chenyao Wang, Mitsuaki Oki, Chihiro Fujii, Takayuki Kondo","doi":"10.5692/clinicalneurol.cn-002246","DOIUrl":"10.5692/clinicalneurol.cn-002246","url":null,"abstract":"<p><p>Duchenne and Becker muscular dystrophies are X-linked disorders that predominantly affect men; however, women can also develop symptomatic dystrophinopathy. We report a case of female dystrophinopathy characterized by childhood-onset motor difficulties, which evolved into slowly progressive, asymmetric muscle weakness in adulthood. The patient was a 79-year-old woman who had difficulty running since childhood but maintained independence in performing activities of daily living for many years. She began to experience gait difficulty at ~62 years of age, with gradually progressive muscle weakness that showed marked asymmetry. During initial evaluation at 68 years of age, skeletal muscle imaging revealed asymmetric muscle involvement suggestive of dystrophinopathy. A muscle biopsy demonstrated dystrophic changes; however, genetic testing was not performed at that time due to a lack of health insurance coverage. Thus, a definitive diagnosis could not be established. Upon further disease progression, the patient was re-evaluated at 79 years of age. On re-assessment, immunohistochemistry of the muscle biopsy revealed a mosaic pattern of dystrophin expression. Subsequent genetic analysis using multiplex ligation-dependent probe amplification identified a heterozygous deletion of exons 45-52 in the dystrophin gene that lead to an ultimate diagnosis of female dystrophinopathy. Clinical and radiological findings of asymmetric muscle distribution was considered characteristic of symptomatic female carriers. The clinical manifestations of dystrophinopathy in women are highly variable, and the absence of an apparent family history often contributes to a delayed diagnosis. The present case highlights the diagnostic challenges associated with female dystrophinopathy and underscores the importance of consideration of this condition in women presenting with progressive muscle weakness, particularly when marked asymmetry is evident. Early recognition and appropriate genetic testing are essential for establishing an accurate diagnosis, clinical management, and long-term follow-up, including surveillance for potential cardiac and central nervous system involvement.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"540-547"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413485","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":"[Successful treatment with rituximab in unilateral relapsing primary CNS vasculitis: a case report].","authors":"Ryo Morikawa, Katsuhiko Kunitake, Junichiro Suzuki, Noriyoshi Nakai, Mari Yoshida, Yasuhiro Ito","doi":"10.5692/clinicalneurol.cn-002262","DOIUrl":"10.5692/clinicalneurol.cn-002262","url":null,"abstract":"<p><p>We report a case of unilateral relapsing primary CNS vasculitis (PCNSV) treated with Rituximab. A 52-year old man initially diagnosed as PCNSV by brain biopsy. He was treated with corticosteroids and no recurrence had been recognized for 4 years. However, after the reduction of corticosteroids to 9 mg, PCNSV recurred many times only in the right hemisphere. Mycophenolate mofetil and cyclophosphamide were not effective. We diagnosed this patient as unilateral relapsing PCNSV (UR-PCNSV), which is resistant to treatment and repeatedly relapses unilaterally. After rituximab was introduced, there was no clinical recurrence and abnormal lesions on head MRI improved. We emphasize that rituximab is a good candidate drug for treating the UR-PCNSV.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"553-558"},"PeriodicalIF":0.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392032","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}