Clinical Neurology最新文献

{"title":"[Is recurrent acute myelopathy without abnormalities on MRI or CSF testing truly caused by an inflammatory process related to MOGAD?]","authors":"Takashi Irioka","doi":"10.5692/clinicalneurol.cn-002293","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002293","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220861","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":"[Is recurrent acute myelopathy without abnormalities on MRI or CSF testing truly caused by an inflammatory process related to MOGAD?]","authors":"Rie Wakatsuki, Tomoyasu Bunai, Kouta Oka, Akiyuki Takenouchi, Kazuki Watanabe, Tomohiko Nakamura","doi":"10.5692/clinicalneurol.cn-002318","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002318","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220848","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":"[Progressive myoclonic epilepsy in two brothers: genetic testing in the younger brother and pathological examination in the elder brother].","authors":"Raita Kikuchi, Kenji Ishihara, Rikiya Furutani, Jun-Ichi Shiota, Mitsuru Kawamura, Mari Yoshida, Yasushi Iwasaki","doi":"10.5692/clinicalneurol.cn-002185","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002185","url":null,"abstract":"<p><p>We report progressive myoclonic epilepsy in two brothers. The younger brother was diagnosed with progressive myoclonic epilepsy type 1 (EPM1) by genetic testing, and the elder brother underwent pathological examination. The parents were cousins. Both brothers presented with epileptic seizures in their early teens, followed by progressive cognitive impairment and cerebellar ataxia. In later life, epileptic seizures became intractable. The elder brother died at age 71, and the younger brother died at age 63. Genetic examination of the younger brother revealed a mutation in the CSTB gene. Pathological examination of the elder brother revealed cerebellar degeneration, mainly characterized by the loss of granule and Purkinje cells, as well as frontotemporal lobe atrophy, findings consistent with EPM1. However, the immunohistochemical findings differed from those in the previously reported case, suggesting that they may reflect a family-specific genetic background.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220847","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":"[Coexistence of progressive supranuclear palsy and idiopathic normal pressure hydrocephalus: pathophysiology and response to shunt surgery].","authors":"Naoki Yamahara, Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-002283","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002283","url":null,"abstract":"<p><p>This review aims to summarize the frequency, pathophysiological features, and therapeutic effectiveness of shunt surgery in cases of coexisting progressive supranuclear palsy (PSP) and idiopathic normal pressure hydrocephalus (iNPH). In this review, we showed that the coexistence of these two disorders is not rare, that they may be related through the glymphatic system, and that in cases clinically suspected of having both conditions, improvement after shunt surgery may persist for one year or more, whereas in six pathologically confirmed cases, the duration of improvement was one year or less. However, the clinical characteristics of shunt-responsive patients among cases clinically suspected of having both conditions remain unclear. To address this issue, we are conducting a multicenter observational study to validate the usefulness of reduced frontal lobe blood flow on cerebral perfusion single-photon emission computed tomography, as previously reported.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220805","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 idiopathic orbital myositis presenting with acute-onset left blepharoptosis].","authors":"Ryota Sugiyama, Tomotaka Mizoguchi, Seiya Jinnouchi, Takayoshi Akimoto, Hideto Nakajima","doi":"10.5692/clinicalneurol.cn-002285","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002285","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220843","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":"Subdural effusion with hydrocephalus in dentatorubral-pallidoluysian atrophy: A case report.","authors":"Hisashi Takahashi, Shunki Yamaguchi, Kengo Tsuji, Kouichi Takano, Takashi Kasai, Tomoyuki Ohara","doi":"10.5692/clinicalneurol.cn-002256","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002256","url":null,"abstract":"<p><p>A 56-year-old woman with dentatorubral-pallidoluysian atrophy (DRPLA) was admitted with progressive disturbance of consciousness. Computed tomography of the head revealed bilateral subdural effusions compressing the cerebral hemispheres. The effusions enlarged despite conservative management, so burr-hole drainage was performed. Unexpectedly, marked hydrocephalus developed postoperatively, resulting in a poor neurological outcome. Subdural effusion with hydrocephalus (SDEH) is a condition that usually occurs secondary to severe head injury or neurosurgical procedures, but reports of its occurrence in association with neurodegenerative disorders are scarce. We speculate that repeated head injuries and periventricular parenchyma damage associated with DRPLA contributed to the development of SDEH. This case highlights the need for awareness of SDEH as a potential complication in neurodegenerative disorders such as DRPLA.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149220839","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 paroxysmal sympathetic hyperactivity following hypoxic brain injury with ‍difficult treatment].","authors":"Sera Tomida, Shuro Kogawa, Hideki Wada, Toshika Hata, Ryosei Nishimura","doi":"10.5692/clinicalneurol.cn-002291","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002291","url":null,"abstract":"<p><p>The patient was a woman in her 20s. In June of a certain year, she experienced cardiopulmonary arrest due to acute epiglottitis and was admitted to the ICU and required mechanical ventilation. From the first day of hospitalization, she exhibited generalized seizures and was diagnosed with acute symptomatic seizures following hypoxic brain injury. Treatment with diazepam and levetiracetam was initiated, leading to symptom resolution. However, around the seventh day of hospitalization, she began experiencing episodic limb muscle hypertonia and fever. Based on diagnostic criteria, she was diagnosed with paroxysmal sympathetic hyperactivity on the 20th day of hospitalization. Although treatment with propranolol, gabapentin, and baclofen was initiated, her symptoms persisted. On the 38th day of hospitalization, morphine hydrochloride and methylprednisolone were administered, resulting in remarkable improvement. The present case shows that accurate diagnosis of paroxysmal sympathetic hyperactivity following hypoxic brain injury, particularly its differentiation from acute symptomatic seizures, is essential for appropriate management.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149070301","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 anti-N-methyl-D-aspartate (NMDA) receptor encephalitis presenting with characteristic symptoms such as altered sense of time, visual distortion and hallucinations].","authors":"Keigo Nakamura, Akihito Koseki, Naoki Morishita, Youji Suzuki, Naoki Sakai, Takahiro Iizuka","doi":"10.5692/clinicalneurol.cn-002181","DOIUrl":"10.5692/clinicalneurol.cn-002181","url":null,"abstract":"<p><p>A previously healthy 20-year-old woman was admitted to our hospital because of psychobehavioral alterations, generalized seizure, and post-ictal drowsiness. Six weeks before this admission, depression developed, followed by dysgeusia, anorexia, hearing loss, auditory hallucinations, logoclonia, and generalized seizures, leading to the first hospitalization. On admission, the temperature was 37°C. She was awake, well oriented, but had logoclonia. Brain MRI and electroencephalography (EEG) were both unremarkable, but cerebrospinal fluid (CSF) analysis revealed 7 cells/μl. After admission, she began to exhibit a variety of psychosomatic symptoms, such as altered sense of time, visual disturbances, visual hallucinations, and phonological paraphasia, and logoclonia. On day 4 she was once discharged home, but 4 days later, she was re-admitted to the hospital because of recurrence of seizure. An EEG recorded on day 1 revealed epileptiform discharges arising from the Cz and Pz. CSF-restricted oligoclonal bands were detected. Because of the suspicion of autoimmune encephalitis, the patient was treated with 3 cycle of intravenous high-dose methylprednisolone and 4 rounds of plasma exchanges, resulting in resolution of seizures; however, cognitive impairment and an altered sense of time persisted. Antibody test results came back positive for GluN1 (estimated CSF antibody titers, 1:4) with neuropil staining on rat brain immunohistochemistry, confirming the diagnosis of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, but no myelin oligodendrocyte glycoprotein antibodies were detected. No ovarian teratoma was found. Following rituximab therapy, cognitive function improved. This case highlights that a forme fruste of anti-NMDAR encephalitis can be attributed to low titers of GluN1 antibodies, and cause a variety of psychosomatic symptoms, without development of typical spectrum of anti-NMDAR encephalitis. Careful assessment is required for appropriate diagnosis and treatment.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"620-628"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702389","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":"[Practice of obstetric medicine in neurological disorders: towards evidence-based shared decision-making (SDM)].","authors":"Mikako Goto","doi":"10.5692/clinicalneurol.cn-002243","DOIUrl":"10.5692/clinicalneurol.cn-002243","url":null,"abstract":"<p><p>Obstetric medicine is a specialized field providing medical care to women with internal diseases who wish to conceive or whose conditions manifest during pregnancy. It bridges the gap between internal medicine and obstetrics by addressing the medical complexities that arise when the physiological changes of pregnancy unmask or exacerbate pre-existing conditions. In clinical practice, Japanese physicians rely heavily on electronic package inserts, which are notably conservative; approximately 21.6% of drugs are contraindicated for pregnant women in Japan, compared to only 4.3% in Australia. This discrepancy arises because Japanese labeling often prioritizes animal developmental toxicity data over human epidemiological evidence. However, these inserts do not have absolute binding force, and clinical judgment based on medical validity should prevail. Since pre-marketing clinical trials for pregnant women are ethically nonexistent, risk assessment relies on post-marketing observational studies. We present evidence from an integrated database of 12,971 pregnancy cases, encompassing findings on various medications such as domperidone, fluoroquinolones, and triptans, among others. Regarding multiple sclerosis (MS) therapies, recent data from the German MS Pregnancy Registry on S1P receptor modulators indicate no increased risk of major malformations but suggest potential fetal toxicity, such as an increased risk of small-for-gestational-age (SGA) infants. Through the Japan Association of Medical Sciences' TEAM project, cross-cutting guidelines have been developed to support patients' reproductive goals. We aim for a society where patients can make informed, autonomous choices through shared decision-making with healthcare providers regarding their treatment and pregnancy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"599-603"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702313","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":"[Tibial nerve somatosensory evoked potentials localize the responsible level in tandem spinal stenosis: a case report].","authors":"Kazuki Sogawa, Tetsuo Ando, Toshio Fukutake, Kotaro Kohara, Motoo Kubota, Masahiro Sonoo","doi":"10.5692/clinicalneurol.cn-002281","DOIUrl":"10.5692/clinicalneurol.cn-002281","url":null,"abstract":"<p><p>An 84-year-old man had experienced unsteadiness while walking and coldness in both lower limbs for about 18 months, with gradual progression. Neurological examination revealed decreased tendon reflexes in the lower extremities, a positive Romberg sign, and a wide-based gait. MRI demonstrated spinal canal stenosis in the cervical and lumbar regions. Because concomitant peripheral neuropathy was suspected, electrophysiological studies were performed. Nerve conduction studies showed no evidence of peripheral neuropathy. Tibial nerve somatosensory evoked potentials (SEP) revealed prolonged N21-P38 interpeak latency and reduced P38 amplitude, suggesting a predominant central conduction disturbance. Based on these findings, thoracic MRI was performed and revealed the most severe spinal cord compression at the T9/10 level. Discectomy and posterior decompression at this level resulted in improvement of gait. Tibial nerve SEP can be useful for identifying the responsible level in tandem spinal stenosis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"648-651"},"PeriodicalIF":0.0,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148702380","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}