Clinical Neurology最新文献

{"title":"[Severe eosinophilic granulomatosis with polyangiitis-related peripheral neuropathy after the cessation of mepolizumab. A case report].","authors":"Tomoki Kawasaki, Akiko Tamura, Masunari Shibata, Kazuto Nishinaka, Satoko Nozato, Fukashi Udaka","doi":"10.5692/clinicalneurol.cn-001992","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-001992","url":null,"abstract":"<p><p>A 78-years-old man was treated for asthma and pansinusitis for >5 years, and mepolizumab was initiated two years previously. Two months after the cessation of mepolizumab treatment, the asthma symptoms worsened and acute progressive muscle weakness and sensory disturbance developed. On day 8 after the onset of weakness and hypoesthesia, the patient presented with complete flaccid tetraplegia and diffuse hypoesthesia of all extremities, without paresthesia or pain, and was admitted to our hospital. Blood tests revealed eosinophilia without anti-neutrophil cytoplasmic antibody elevation. Nerve conduction studies revealed severe axonal polyneuropathy and multifocal absent F-waves. Cerebrospinal fluid was normal. Eosinophilic granulomatosis with polyangiitis (EGPA) and Guillain-Barré syndrome (GBS) were suspected, and high-dose methylprednisolone was administered, followed by oral prednisolone. Eosinophils rapidly disappeared; however, the neurological symptoms did not improve. On day 16, sural nerve biopsy revealed myelinated fiber loss in most of the fibers in every nerve bundle regardless of fiber size, while eosinophilic infiltration in the epineurium and findings suggestive of necrotizing vasculitis were not observed. The results did not fulfill the pathological criteria for EGPA but supported the changes in vasculitis; hence, EGPA was diagnosed. Intravenous immunoglobulin, azathioprine, and rituximab were administered, and the prednisolone dose was gradually reduced to 10 ‍mg/d. The eosinophil count increased to 50/μl without pneumonia recurrence or worsening asthma. Neuropathy in the upper limbs gradually improved over two years, whereas that in the lower limbs did not change. This is the first reported case of sequential exacerbation of asthma and onset of EGPA after mepolizumab discontinuation. Among patients with asthma, the cessation of mepolizumab treatment may lead to the development of EGPA with an atypical clinical course, such as rapidly progressive severe neuropathy mimicking GBS.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143068567","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-ganglioside antibody-positive Guillain-Barré syndrome with asymmetrical muscle weakness throughout the course of the disease].","authors":"Hitomi Sato, Rena Okudera, Yu Hongo, Taro Matsui, Katsunori Ikewaki, Kazushi Suzuki","doi":"10.5692/clinicalneurol.cn-002001","DOIUrl":"10.5692/clinicalneurol.cn-002001","url":null,"abstract":"<p><p>A 56-year-old woman who presented with left drop foot and low back pain a week after the onset of diarrhea. Neurological symptoms progressed for a week and gradually improved thereafter. No weakness was observed in upper limbs and clearly asymmetrical muscle weakness was observed in left lower limbs during the course of the disease. Nerve conduction study demonstrated absent motor responses in the left tibial and fibular nerves, and compound muscle action potentials in the right tibial nerve was decreased in amplitude without conduction slowing. Serum IgG anti-GalNAc-GD1a antibody and anti-ganglioside complex antibodies were positive. Based on these findings, we diagnosed her as a rare variant of Guillain-Barré syndrome (GBS) with marked asymmetrical muscle weakness. In the literature, GBS patients with asymmetrical muscle weakness often have anti-ganglioside antibodies associated with acute motor axonal neuropathy. A detailed history taking and information on the clinical course are helpful for accurate diagnosis of GBS with atypical distribution of weakness.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"27-31"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142855977","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":"[Summarising a case you experienced: how to write an abstract effectively].","authors":"Akiyuki Hiraga","doi":"10.5692/clinicalneurol.cn-002034","DOIUrl":"10.5692/clinicalneurol.cn-002034","url":null,"abstract":"<p><p>Just like the title, a well-written abstract is crucial for submitting your case report to conferences or medical journals. Authors should write the abstract following four key principles. First, one should be guided by the ABCs of writing-accurate, brief, and clear. Do not include unnecessary information. Second, the abstract is not the preview. Abstracts should be stand-alone. Do not write 'we report this case, adding discussion with the literature' or 'additional cases are needed in future' at the end of the abstract. Third, do not simply focus on the rarity of the case. Avoid writing statements such as 'we report this case because it is a rare, valuable/worthful case'. The abstract of a case needs a clear 'learning point' for readers. Lastly, carefully confirm and follow the guidelines of target conferences or journals.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"48-52"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142877130","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 L-2-hydroxyglutaric aciduria diagnosed with involuntary movements, in which improvement in motor symptoms was achieved following treatment].","authors":"Masaya Shimozato, Takeo Sakurai, Tomonori Yaguchi, Takayoshi Shimohata, Hiroshi Nishida","doi":"10.5692/clinicalneurol.cn-002026","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002026","url":null,"abstract":"<p><p>A 49-year-old female presented with the primary complaint of hand tremors. Neurological examination on admission revealed signs of cognitive impairment, bulbar palsy, dystonia, cerebellar ataxia, and pyramidal tract disease. T₂-weighted brain MRI revealed hyperintense signals in the subcortical white matter, basal ganglia, and cerebellar dentate nucleus, with no atrophy of the brainstem or corpus callosum. Urinary organic acid analysis revealed elevated 2-hydroxyglutaric acid levels. Although the optical isomers could not be distinguished, L-2-hydroxyglutaric aciduria was diagnosed based on the disease course, symptoms, and characteristic MRI findings. The patient was started on riboflavin-enriched compounds and levocarnitine, resulting in an improvement in the Scale for the Assessment and Rating of Ataxia (SARA) score from 21 to 15 after six months. The case suggests that symptoms in adult patients who have not been treated for a long time can be improved by appropriate diagnosis based on neurological presentation, characteristic MRI findings, and intervention.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143068563","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":"[How to make effective figures in case reports].","authors":"Akiyuki Hiraga","doi":"10.5692/clinicalneurol.cn-002044","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002044","url":null,"abstract":"<p><p>Figures are essential components of case reports, often conveying information more effectively than the text. Common figure types include images, pathology slides, photographs, schematic drawings, and clinical courses. Each figure type should follow four design principles: alignment, repetition, proximity, and contrast. Precise \"alignment\" of elements and \"repetition\" with font usage are crucial for improving clarity. Employing \"contrast,\" such as arrows to highlight specific areas, can also significantly improve visual impact. Additionally, enlarged views or detailed schematics can be beneficial in certain cases. Creating figures early in the process can boost your motivation to write case reports. The enjoyment of writing case reports lies in discovering the key learning points of the case and summarising them. Additionally, creating figures for your case can be enjoyable.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143068566","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":"[Rehabilitation therapy for aquaporin-4 antibody positive neuromyelitis optica spectrum disorders].","authors":"Junko Ikeda, Kazuki Muguruma, Kazuhide Ochi, Satomi Kushitani, Yumiko Kaseda, Hirofumi Maruyama","doi":"10.5692/clinicalneurol.cn-001993","DOIUrl":"10.5692/clinicalneurol.cn-001993","url":null,"abstract":"<p><p>Forty-five cases with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders (NMOSD) who underwent convalescent rehabilitation were studied. After excluding three cases with recurrence during rehabilitation treatment, the Expanded Disability Status Scale of Kurtzke improved a median of 1 point. Corticosteroids were the most used disease-modifying drugs (DMDs). Three cases relapsed during rehabilitation treatment. Six cases developed febrile infections. Thirty-four cases were discharged home, but half of the cases in their 80s were transferred to a medical care hospital. In the rehabilitation treatment of NMOSD, reducing the risk of recurrence by appropriate DMDs and preventing infections are important. Information sharing using a regional collaborative medical care plan is useful.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"16-21"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142877129","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":"[How to use clinical neurophysiology in functional neurological disorders (FND)?]","authors":"Yoshikazu Ugawa","doi":"10.5692/clinicalneurol.cn-002050","DOIUrl":"10.5692/clinicalneurol.cn-002050","url":null,"abstract":"<p><p>I have reviewed the utility of clinical neurophysiological examinations in recently highlighted functional neurological disorders (FND) focusing mainly on functional movement disorders (FMD). There are many neurophysiological methods useful for diagnosis of FMD. I will hereafter summarize a few of them in the following part. Surface EMG: This is one of minimally required examinations for clinical analysis of movement disorders. It plays roles in the exclusion of organic disorders and showing positive findings to support FND, especially for functional tremor. The power spectral analysis of surface EMG clearly proves a few useful findings, such as entrainment, distraction and others. Somatosensory evoked potential (SEP): Giant SEP is critical because it proves organic disorders with hyperexcitability of the sensory cortex. Single pulse transcranial magnetic stimulation (TMS): Normal motor evoked potential (MEP) showing intact corticospinal tracts is a positive finding for functional paresis patients. It is also useful to exclude the corticospinal tracts organic dysfunction. Jerk-locked back averaging (JLA), bereitschaftspotential (BP), event related desynchronization (ERD): These are sometimes used for functional disorders, but their clinical validity remains to be determined.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142878229","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 dysarthria and ataxia appeared late after treatment of myelin oligodendrocyte glycoprotein (MOG) antibody-related isolated rhombencephalitis].","authors":"Tosi Sai, Keisuke Imai, Takehiro Yamada, Masanori Cho, Atsushi Yamamoto, Testuya Ioku","doi":"10.5692/clinicalneurol.cn-002031","DOIUrl":"10.5692/clinicalneurol.cn-002031","url":null,"abstract":"<p><p>A 49-year-old female was admitted to our hospital due to acute-onset dysarthria and unstable gait. Brain MR diffusion weighted imaging revealed high signal intensities in the midbrain. Brain tumors, abscess and demyelinating lesions was suspected firstly as etiology of the lesion and antibiotics and antiviral drug were started under consulting with the neurosurgeon about brain biopsy. However, despite these treatment her symptoms were deteriorated gradually and intravenous high dose methylprednisolone was added. Since starting this treatment, her symptoms had been improving and the same treatment was repeated once and oral administration of prednisolone (0.5 ‍mg/kg/day) was started. Based on the positive for anti myelin oligodendrocyte glycoprotein (MOG) antibody in the serum, she was ultimately diagnosed with MOG related rhombencephalitis and discharged to her home on the 45th day. After discharge, new paroxysmal symptoms such as paroxysmal dysarthria and ataxia (PDA) had appeared whose symptoms occurred several times a day and lasted for a few seconds to several tens of seconds. These symptoms were regarded as PDA secondary to sequelae of MOG related rhombencephalitis and oral administration of Carbamazepine was started. After the treatment, the symptoms completely disappeared. The differential diagnosis of midbrain tegmental lesions should consider MOG antibody-associated disease, and in cases where delayed-onset PDA occurs, carbamazepine may be effective.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"45-47"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142855978","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":"[Posterior reversible encephalopathy syndrome after COVID-19 in a patient with chronic renal failure].","authors":"Masato Kochi, Yoshiki Yokoyama","doi":"10.5692/clinicalneurol.cn-002013","DOIUrl":"10.5692/clinicalneurol.cn-002013","url":null,"abstract":"<p><p>A 61-year-old man with chronic renal failure had an embolic stroke of undetermined source that was treated with warfarin. Five weeks later, the patient contracted coronavirus disease (COVID-19). Six days after the onset of COVID-19, high blood pressure (>200 ‍mmHg) and consciousness disturbance were reported. CT demonstrated symmetrical hypodensity areas in the bilateral cerebellar hemispheres. MRI revealed hyperintensity lesions in the bilateral cerebellar hemispheres and pons on the T₂-weighted and fluid-attenuated inversion recovery images. Moreover, cerebellar lesions appeared as hyperintensity areas on apparent diffusion coefficient mapping. Based on these findings, a diagnosis of posterior reversible encephalopathy syndrome (PRES) was made. The patient was treated with antihypertensive drugs, and the consciousness level improved gradually. MRI after one month showed that the lesions had disappeared. PRES should be considered if the brain CT of patients with COVID-19 shows a low-density lesion, especially in patients with risk factors for PRES such as chronic renal failure or hypertension.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"32-38"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142877083","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":"[Interictal burden of migraine and its evaluations: a literature review].","authors":"Tsubasa Takizawa, Ryo Takemura, Hisaka Igarashi, Yasuhiko Matsumori, Minako Sato, Kaname Ueda","doi":"10.5692/clinicalneurol.cn-002016","DOIUrl":"10.5692/clinicalneurol.cn-002016","url":null,"abstract":"<p><p>We conducted a targeted literature review on patient burden during the interictal period of migraine. The results of the literature review revealed that: (1) migraine-associated burden persists into the interictal period and is not confined to the headache episodes themselves; (2) anxiety over the possible recurrence of headache episodes is a factor that reduces daily activities and quality of life (QOL); and (3) prophylactic treatment with calcitonin gene-related peptide (CGRP) antibody drugs may reduce the burden during the interictal period. From these findings, it is considered important in migraine treatment to identify the unmet needs of patients, including the burden during the interictal period, and to select an appropriate treatment method based on the burden experienced by individual patients.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"8-15"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142878230","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}