Clinical NeurologyPub Date : 2025-06-21DOI: 10.5692/clinicalneurol.cn-002064
Minori Sawada, Gaku Okumura, Ken Takasone, Nagaaki Katoh, Yoshiki Sekijima
{"title":"[A case of POEMS syndrome presenting craniocervical vascular stenosis].","authors":"Minori Sawada, Gaku Okumura, Ken Takasone, Nagaaki Katoh, Yoshiki Sekijima","doi":"10.5692/clinicalneurol.cn-002064","DOIUrl":"10.5692/clinicalneurol.cn-002064","url":null,"abstract":"<p><p>A 54-years-old Japanese man visited local hospital with six-month history of progressive numbness and muscle weakness in his lower limbs. He was diagnosed with POEMS syndrome based on positive serum M-protein, elevated serum VEGF, and splenomegaly. MRI showed multiple cerebral infarcts in the watershed area, and MRA showed complete vascular occlusion of the right internal carotid artery and severe vascular stenosis of the left common carotid artery, suggesting hemodynamic cerebral infarction. He was referred to our department and treated with three courses of Daratumumab-Bortezomib-Dexamethasone (DBd) therapy. After normalization of VEGF was confirmed, right superficial temporal artery-middle cerebral bypass surgery was performed. He has been kept in good condition with improved muscle strength and walking ability, and normalized VEGF level under the maintenance Daratumumab-Lenalidomide-Dexamethasone (DLd) therapy. Because POEMS syndrome-associated vasculopathy can develop even in the very early stage of the disease, intensive evaluation of craniocervical vessels before treatment initiation is very important for treatment selection and risk assessment.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369324","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}
Clinical NeurologyPub Date : 2025-06-21DOI: 10.5692/clinicalneurol.cn-002115
Fujio Umehara
{"title":"[Remarkable improvement of metastatic occipital condyle syndrome in a patient with lung adenocaricinoma treated with osimertinib].","authors":"Fujio Umehara","doi":"10.5692/clinicalneurol.cn-002115","DOIUrl":"10.5692/clinicalneurol.cn-002115","url":null,"abstract":"<p><p>A woman in her 50s. Since October of X-1, she had been suffering from lower back and occipital pain. Despite undergoing medical evaluations at multiple hospitals, no discernible abnormalities were identified. As her symptoms worsened, she presented to our department in January of X, reporting severe pain in the left occipital to posterior neck that increased with both neck flexion and extension. A neurological examination revealed a left-sided deviation of the tongue (left hypoglossal nerve paralysis). A head MRI revealed signal abnormalities in the left occipital condyle and multiple nodules with contrast effects in the brain parenchyma. A computed tomography (CT) scan revealed a mass in the left lung, multiple intrahepatic masses, and bone destruction in the spine and left occipital condyle. In light of these findings, a diagnosis of lung cancer with metastasis to multiple organs and occipital condyle syndrome due to metastasis to the left occipital condyle was suspected. Subsequent cytological analysis of bronchoalveolar lavage fluid and liver biopsy substantiated the diagnosis of adenocaricinoma. The subsequent administration of osimertinib, an epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor, led to a reduction in the size of the tumor, as well as improvements in the hypoglossal nerve palsy and occipital pain. It is imperative to note that occipital condyle syndrome signifies the metastasis of a malignancy to the base of the skull, necessitating meticulous observation and management.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369328","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 severe COVID-19 infection with multiple brain microbleeds spreading to the subcortical white matter, internal capsule and splenium of the corpus callosum].","authors":"Shoko Aburatani, Eito Miura, Yoshitaka Umeda, Shimon Aoki, Nobuya Fujita, Mutsuo Oyake","doi":"10.5692/clinicalneurol.cn-002072","DOIUrl":"10.5692/clinicalneurol.cn-002072","url":null,"abstract":"<p><p>A 23-year-old female student was admitted to a local hospital because of consciousness disturbance caused by diabetic ketoacidosis on the 4th day after COVID-19 infection. She was subsequently transferred to our hospital, having developed respiratory failure, acute renal failure and DIC. Although intensive treatment improved her condition, she remained hypoactive. Brain MRI revealed multiple microbleeds (MBs) spreading to the subcortical white matter, internal capsule, splenium of the corpus callosum and brainstem. The WAIS-IV score suggested general attention deficit disorder and mild impairment of working memory and processing speed. These symptoms disappeared with time, and she was able to return to her studies without any after-effects. COVID-19 tends to cause thrombosis and MBs in the brain due to vascular endothelial damage. Although the reason for the specific localization of these MBs remains unclear, differences in regional vulnerability to cytokines may have been partly responsible.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369326","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":"[Questionnaire survey regarding pediatric to adult transitional medical care targeting members of Japanese Society of Neurology].","authors":"Toshio Saito, Yoshio Sakiyama, Katsuhisa Ogata, Yoko Mochizuki, Ichiro Yabe, Hideki Mochizuki","doi":"10.5692/clinicalneurol.cn-002055","DOIUrl":"10.5692/clinicalneurol.cn-002055","url":null,"abstract":"<p><p>The Special Committee on Measures for Transition from Pediatric to Adult Health Care surveyed approximately 9,000 members of the Japanese Society of Neurology regarding transitional care. Only 744 responses were returned, less than 10% of the total number of members contacted. More than half answered that they generally provide treatment for adult patients with a childhood-onset disease, with many noting as reasons that the related diseases and ages in such cases are targeted by neurology specialists, and that other adult medical departments are not equipped to treat them. As for reasons given for not treating such patients, lack of knowledge related to developmental disorders, lack of support system, and difficulties with communication were noted. There were no noticeable differences in the responses of providing treatment for adult patients with a childhood-onset disease in association with the affiliated regional association or branch. These results indicate that resolution of issues related to neurologist unfamiliarity with pediatric neurological disorders and lack of relevant information are important issues to be addressed for establishment of a smooth medical care transition.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369327","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}
Clinical NeurologyPub Date : 2025-06-21DOI: 10.5692/clinicalneurol.cn-002119
Fujio Umehara
{"title":"[A case of rapidly progressive HTLV-I associated myelopathy that developed after a living donor kidney transplantation].","authors":"Fujio Umehara","doi":"10.5692/clinicalneurol.cn-002119","DOIUrl":"10.5692/clinicalneurol.cn-002119","url":null,"abstract":"<p><p>Case Male in his 40s. Due to end-stage renal failure, a living donor kidney transplantation was performed in September 2019 from his mother, who was positive for anti-HTLV-1 antibodies, to this case, who was negative for anti-HTLV-1. He was admitted in September 2021 due to progressive lower limb muscle weakness since March 2021. The patient was unable to stand or walk due to spastic paraplegia. Spinal cord MRI T<sub>2</sub>-weighted image showed continuous high signal and myelopathy from the cervical to thoracic spinal cord. Serum and spinal fluid anti-HTLV-1 antibodies were positive. The diagnosis of rapidly progressive HTLV-1-associated myelopathy (HAM) was made, which was problematic because kidney transplantation from an HTLV-1-positive donor to a negative recipient was performed in this case, even though it had already been noted that living donor-negative recipients are at high risk of developing HAM.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369325","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}
Clinical NeurologyPub Date : 2025-05-27Epub Date: 2025-04-25DOI: 10.5692/clinicalneurol.cn-002070
Daisuke Danno, Paul Shanahan, Manjit Matharu
{"title":"Clinical characteristics of hemiplegic migraine: a clinical study of 163 cases in a tertiary care headache centre.","authors":"Daisuke Danno, Paul Shanahan, Manjit Matharu","doi":"10.5692/clinicalneurol.cn-002070","DOIUrl":"10.5692/clinicalneurol.cn-002070","url":null,"abstract":"<p><strong>Objective: </strong>Although population-based studies of hemiplegic migraine (HM) exist, large-scale clinic-based studies focusing on the detailed clinical characteristics of HM have not been reported. This study aims to define the clinical characteristics of HM in a tertiary care headache centre.</p><p><strong>Methods: </strong>A retrospective analysis was conducted based on the medical records of HM patients.</p><p><strong>Patients: </strong>This study included 163 consecutive HM patients who visited the National Hospital for Neurology and Neurosurgery between 2006 and 2013.</p><p><strong>Results: </strong>According to the diagnostic criteria of International Classification of Headache Disorders (ICHD-3β), 142 patients were diagnosed with HM. Although 21 patients did not satisfy the diagnostic criteria, migrainous headaches with repetitive hemiparesis were reported and other disorders were excluded, hence these patients were clinically diagnosed with HM. The temporal progression of aura symptoms was atypical in 40 patients. The median duration of hemiparesis was 24 hours (interquartile range: 3-60 hours) which was far longer than that of previous population-based studies. The lifetime experience of an episode of motor aura exceeding 72 hours was reported in 51.6%. Hemiparesis was observed without full recovery in 9 patients (5.5%).</p><p><strong>Conclusions: </strong>In many HM patients, the temporal progression of aura symptoms was diverse compared to typical descriptions in the literature, and the aura symptoms sustained longer than reported in the population-based study.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"338-351"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144031711","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}
Clinical NeurologyPub Date : 2025-05-27Epub Date: 2025-05-09DOI: 10.5692/clinicalneurol.cn-002095
Masami Tanaka
{"title":"[Current trends in multiple sclerosis/neuromyelitis optica spectrum disorder therapy: considerations for domestic administration and personalized therapy].","authors":"Masami Tanaka","doi":"10.5692/clinicalneurol.cn-002095","DOIUrl":"10.5692/clinicalneurol.cn-002095","url":null,"abstract":"<p><p>In multiple sclerosis and neuromyelitis optica spectrum disorders, the risk of relapse can no longer be reduced to nearly zero. Consequently, the possibility of misdiagnosis must be considered when a relapse occurs. Nevertheless, managing these conditions requires efforts to minimize the risk of infection and avoid overtreatment. Looking ahead, future treatments may focus not only on enhancing myelin regeneration but also on addressing neuronal and axonal degeneration. Additionally, immune reconstitution therapy-designed to reset and rebuild the immune system's memory-may become a viable option. As such, it will be increasingly important to avoid excessive dosages and to tailor personalized therapies to meet the specific needs of each patient.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"319-330"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144003334","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":"[Intravenous glucose infusion may have caused refeeding syndrome in a patient with advanced amyotrophic lateral sclerosis].","authors":"Naoki Yamahara, Nobuaki Yoshikura, Iwasa Yuhei, Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-002086","DOIUrl":"10.5692/clinicalneurol.cn-002086","url":null,"abstract":"<p><p>We present the case of a 69-year-old woman who underwent tracheostomy for advanced amyotrophic lateral sclerosis. The patient was treated with furosemide for leg edema. Body mass index was stable at 21.5 kg/m<sup>2</sup>. The patient was admitted to our hospital after vomiting because of biliary infection. Fluid therapy with 286 kcal/day of glucose was administered, followed by acute deterioration, including tachycardia (120 bpm), glucose intolerance, abdominal pain, hypophosphatemia (required intravenous phosphate supply; 60 mmol/day), and hypokalemia (required intravenous potassium supply; 60 mEq/day). Refeeding syndrome was suspected, and the patient recovered with adjustments in serum electrolyte levels. We demonstrated that glucose infusion can cause refeeding syndrome in patients with advanced amyotrophic lateral sclerosis without low nutritional intake.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"372-375"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144055323","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":"[Factors associated with fatigue in patients with Parkinson's disease].","authors":"Kanako Kurihara, Takayasu Mishima, Koichi Nagaki, Yoshio Tsuboi","doi":"10.5692/clinicalneurol.cn-002061","DOIUrl":"10.5692/clinicalneurol.cn-002061","url":null,"abstract":"<p><p>Fatigue is one of the most frequent non-motor symptoms associated with people with Parkinson's disease (PwPD). In this study, we investigated the relationship between fatigue and patients' background characteristics, disease severity, motor and non-motor symptoms, and cognitive and psychological assessments in PwPD. A total of 80 PwPD were included in this study, 40% of whom experienced fatigue. PwPD with fatigue were associated with being female, dyskinesia, and higher levels of levodopa equivalent daily dose, as well as increased severity of depression, apathy and sleep disturbances, in addition to lower quality of life. Fatigue in PwPD is a symptom that warrants clinical attention, as it is linked to multiple risk factors, indicating the need for further intervention studies that incorporate the perspective of personalized medicine.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"331-337"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144003168","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":"[Early-onset cerebral infarction with psoriasis vulgaris and thromboangiitis obliterans as an athterosclerotic risk factor: a case report].","authors":"Yasunobu Inagaki, Soichiro Abe, Hiroyuki Ishiyama, Takeshi Yoshimoto, Manabu Inoue, Masafumi Ihara","doi":"10.5692/clinicalneurol.cn-002027","DOIUrl":"10.5692/clinicalneurol.cn-002027","url":null,"abstract":"<p><p>The patient is a 45-year-old male. He has psoriasis vulgaris and thromboangiitis obliterans (TAO) as a comorbidity and was transferred to our hospital with dysarthria and right hemiparesis. On arrival, he presented with right hemispatial neglect, hemiparesis, and sensory disturbance. Head MRI showed scattered infarctions at the left middle cerebral artery territory, and 3D TOF MRA showed left middle cerebral artery occlusion. We performed mechanical thrombectomy with effective recanalization despite remained residual stenosis. Dual antiplatelet therapy was initiated, and neurological findings gradually improved. No apparent embolic source was identified including antibodies for vasculitis. He was diagnosed with large artery atherosclerosis with psoriasis and TAO as a vascular risk factor. Psoriasis is known to cause atherosclerosis and inflammatory disease by increasing cardiovascular risk. Contrast-enhanced MRI after three months of treatment for psoriasis showed decreased contrast signal at the stenotic lesion.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"359-365"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144052609","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}