Neurology and Clinical Neuroscience最新文献

{"title":"Lenalidomide‐induced posterior reversible encephalopathy syndrome: A case report","authors":"Marc S. Ayoub, Ahmad Ballout, Joshua Burshtein, Aaron Burshtein, Amanda Lin, David Ledoux","doi":"10.1111/ncn3.12814","DOIUrl":"https://doi.org/10.1111/ncn3.12814","url":null,"abstract":"Posterior reversible encephalopathy syndrome (PRES) is a disorder of cerebrovascular dysregulation. To our knowledge, we present the first case of lenalidomide‐induced PRES in English. A 59‐year‐old woman with history of multiple myeloma status post stem cell transplant and induction triple therapy 6 months ago with partial remission and subsequent high‐dose melphalan 1 month ago presented with progressive vision loss for 1 week. Her systolic blood pressure was >180 mmHg, and neurological examination demonstrated decreased vision bilaterally. Brain MRI revealed significant T2 hyperintensity restricted to temporal and occipital lobes and left occipital parenchymal hemorrhage and diffuse leptomeningeal enhancement. She had a witnessed generalized tonic–clonic seizure and EEG that captured an electrographic seizure originating from the occipital lobe. Her vision improved rapidly after discontinuing the lenalidomide. She was discharged to rehab with a normal neurological examination. We report the first English case of presumed lenalidomide induced PRES in a patient undergoing multiple myeloma treatment.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140711487","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":"Pattern of paresis in amyotrophic lateral sclerosis: A cohort study","authors":"Nan Hu, Lei Zhang, D. Shen, Xun-zhe Yang, Mingsheng Liu, Liying Cui","doi":"10.1111/ncn3.12818","DOIUrl":"https://doi.org/10.1111/ncn3.12818","url":null,"abstract":"To explored the dynamic changes of split phenomenon in multiple antagonistic muscle groups among amyotrophic lateral sclerosis (ALS) patients.One hundred and twenty limb‐onset ALS patients were followed up regularly with an interval of 3 months. Two clinicians collected the muscle strength of elbow extensor‐flexor, wrist flexor‐extensor, knee extensor‐flexor and ankle flexor‐extensor individually.Significant differences in muscles strength were observed between flexors and extensors of elbow, wrist, knee, and ankle (p < 0.05). The frequency of split phenomenon of elbow extensor‐flexor was 15.1%, 13.8%, 19.1%, 17.8%, and 12.5% at the baseline, 3‐, 6‐, 9‐, and 12‐month follow‐up points, respectively. For wrist flexor‐extensor, the frequency was 20.8%, 18.2%, 18.6%, 19.9%, and 20.4%, respectively. For knee extensor‐flexor, the frequency was 3.0%, 8.0%, 7.6%, 8.4%, and 8.9%, respectively. For ankle flexor‐extensor, the frequency was 8.8%, 9.2%, 10.9%, 15.4%, and 15.7%, respectively. For muscles with 3–4 levels of muscle strength, significant dissociation of muscle strength was common, especially among patients with a slow progression rate.Split phenomenon of muscle strength between antagonistic muscles was common in limb‐onset ALS patients. Muscle strength and progression rate were influence factors of the split phenomenon. As disease progressed, the split phenomenon gradually became obvious, and might then slowly disappeared due to the loss of LMNs.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140718397","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":"Usefulness of combined telemedicine for self‐injection of calcitonin gene‐related peptide pathway targeting monoclonal antibodies","authors":"Takao Takeshima, Naoko Sanno, Yumiko Miura, Y. Matsumori","doi":"10.1111/ncn3.12815","DOIUrl":"https://doi.org/10.1111/ncn3.12815","url":null,"abstract":"We examined whether the combined use of telemedicine and self‐injection could improve patient satisfaction.For patients with migraine, the use of calcitonin gene‐related peptide ‐pathway targeting monoclonal antibodies is associated with concerns, such as the burden of doctor visits and anxiety around self‐injection.At four institutions, patients with migraines who used self‐injectable calcitonin gene‐related peptide ‐pathway targeting monoclonal antibodies attempted telemedicine, and web‐based questionnaires and interviews were conducted thereafter.Of the 26 participants, 58% felt that telemedicine helped with self‐injections and 65% wanted to continue using the combination. The average time required per doctor visit was reduced from 158 to 31 min, and the median from 86 to 18 min. The benefits included reduced burden (46%), ease of schedule adjustment (35%), and lower anxiety levels (19%).Although there are issues with telemedicine usage, including the burden on healthcare professionals or lower reimbursement points, the combination of self‐injection of calcitonin gene‐related peptide ‐pathway targeting monoclonal antibodies and telemedicine is effective in improving the experience of patients have migraines.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140735054","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":"Mobile aortic mural thrombus: A rare but important cause of embolic stroke","authors":"Miyu Usui, Shuya Hirano, Shunich Okada, Tomoaki Kameda, T. Kawakami, Akira Sugaya, Kei Aizawa, Koji Kawahito, Shigeru Fujimoto, Ryota Tanaka","doi":"10.1111/ncn3.12817","DOIUrl":"https://doi.org/10.1111/ncn3.12817","url":null,"abstract":"Aortic mural thrombus is a rare but important cause of embolic stroke. We report a case of embolic stroke by mobile aortic mural thrombus discuss the acute management and review the literature. A 50‐year‐old man presented sudden onset right hemiparesis and diffusion‐weighted imaging (DWI) of MRI revealed acute embolic stroke. He showed minimum vascular risk factors and no embolic sources. Transesophageal echocardiography (TEE) performed at 5 days after admission showed mobile floating mass lesion in the ascending aorta (19.3 mm × 8.94 mm). He received surgical thrombectomy to prevent further thromboembolic events and the pathological examination showed thrombotic tissue without malignant findings. Mobile aortic mural thrombus is rare but important cause of embolic stroke. Physicians should evaluate embolic sources using TEE as soon as possible in case of cryptogenic stroke in order to exclude critical condition.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140738604","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":"Challenging neurological presentation: Scrub typhus with meningoencephalitis and multiple cranial nerve involvement","authors":"Uttam Biswas, M. León-Ruiz, Alamgir Shaikh, Prasenjit Das, Shyamal Kanti Pal, R. Ghosh, Julián Benito-León","doi":"10.1111/ncn3.12819","DOIUrl":"https://doi.org/10.1111/ncn3.12819","url":null,"abstract":"A 40‐year‐old male farmer from rural West Bengal, India, presented with fever, headaches, seizure, eschar, and palsy of left cranial nerves VII, VIII, IX, and X as a rare clinical manifestation of scrub typhus. Positive IgM enzyme‐linked immunosorbent assay tests in serum and cerebrospinal fluid confirmed the diagnosis, with brain magnetic resonance imaging showing meningoencephalitis findings. Treatment with steroids and doxycycline led to neurological improvement. This case highlights the need for heightened clinician awareness toward early detection of scrub typhus's atypical presentations to ensure timely and effective treatment.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140740583","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":"Long‐term outcome following magnetic resonance imaging‐guided focused ultrasound unilateral thalamotomy for medication‐refractory essential tremor","authors":"Hisashi Ito, Kazuaki Yamamoto, Shigeru Fukutake, T. Kamei, Toshio Yamaguchi, Takaomi Taira","doi":"10.1111/ncn3.12805","DOIUrl":"https://doi.org/10.1111/ncn3.12805","url":null,"abstract":"To investigate the long‐term outcome 5 years after transcranial magnetic resonance imaging‐guided focused ultrasound (MRgFUS) unilateral ventral intermediate nucleus (Vim) thalamotomy for essential tremor (ET), following our interim reports.This was a prospective and open‐labeled study involving a single center. Ten medication‐refractory ET patients (8 men and 2 women, aged 67.1 ± 17.5 years, all right‐handed) underwent left MRgFUS Vim thalamotomy. They were assessed using the Clinical Rating Scale for Tremor (CRST) and the overall health and quality of life (QOL) scale of the Quality of Life in Essential Tremor Questionnaire (QUEST). Additionally, we assessed safety issues during the study period.Right‐hand tremor improved immediately after the procedure in all patients. The right‐hand CRST score remained significantly improved by 60.2% and 33.5% from baseline at both 1‐ and 5‐years post‐treatment, respectively (both p < 0.05). On the other hand, the left‐hand CRST score and the overall QOL score of QUEST showed no improvement. Adverse events during and immediately after the procedure were mild and reversible. We observed no delayed adverse events.MRgFUS unilateral Vim thalamotomy is adopted as one of the therapeutic options for intractable ET. Further investigation of the methodology for accurate targeting and sonication is important to improve long‐term outcomes involving a larger number of patients. In addition, not only unilateral but also bilateral Vim thalamotomy may be necessary to achieve a better quality of life.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140787408","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":"The Japan MSA registry: A multicenter cohort study of multiple system atrophy","authors":"Ayaka Chikada, Kenta Orimo, J. Mitsui, T. Matsukawa, H. Ishiura, Tatsushi Toda, Hidehiro Mizusawa, Yuji Takahashi, M. Katsuno, K. Hara, Osamu Onodera, Tomohiko Ishihara, Masayoshi Tada, Satoshi Kuwabara, A. Sugiyama, Y. Yamanaka, Ryosuke Takahashi, N. Sawamoto, Yusuke Sakato, T. Ishimoto, Ritsuko Hanajima, Yasuhiro Watanabe, Hiroshi Takigawa, T. Adachi, Koji Abe, Toru Yamashita, Hiroshi Takashima, K. Higashi, Junichi Kira, Ichiro Yabe, Masaaki Matsushima, Katsuhisa Ogata, K. Ishikawa, Y. Nishida, T. Ishiguro, K. Ozaki, Tetsuya Nagata, Shoji Tsuji","doi":"10.1111/ncn3.12809","DOIUrl":"https://doi.org/10.1111/ncn3.12809","url":null,"abstract":"Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure and various motor symptoms. While MSA‐C (cerebellar type) predominates in East Asia, MSA‐P (parkinsonian type) predominates in Europe and North America. This nationwide patient registry aimed to (1) conduct a prospective natural history study of MSA in Japan, (2) facilitate patient recruitment for clinical trials, and (3) deposit bioresources and clinical information in a biobank.Thirteen institutions participated in this study. Clinical information was obtained by neurologists from the patients visiting the hospital every 12 months to assess the UMSARS Part 2 scores and by telephone interviews by nurses every 6 months to assess UMSARS Part 1 scores and to determine whether clinical events had occurred.Demographic data from 329 MSA patients (216 MSA‐C and 113 MSA‐P) were analyzed. The mean age at symptom onset was 58.2 years (standard deviation, 8.9); the mean duration of symptoms at enrollment was 3.5 years (standard deviation, 2.2). The mean 12‐month changes in the UMSARS Part 1 and Part 2 scores were 7.9 (standard deviation, 5.6) and 6.4 (standard deviation, 5.9), respectively. The patient registry proved useful in recruiting participants for clinical trials, including those with gene variants. Clinical information and biospecimens were deposited in a biobank.The study highlighted the importance of telephone interviews in minimizing drop‐out rates in natural history studies and demonstrated similar MSA progression rates across populations. The deposited bioresources are available to researchers upon request, aiming to contribute to future MSA researches.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140366761","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 methylprednisolone therapy for ocular myasthenia gravis: A retrospective study","authors":"S. Yorozu, Jinsoo Koh, Katsuichi Miyamoto, M. Sakata, Y. Nakayama, Maiko Takahashi, Megumi Mori, Junko Taruya, Takuya Matsumoto, Mai Minamino, Shuhei Ogami, Mayako Niwa, Y. Hiwatani, Y. Kajimoto, H. Ishiguchi, Hidefumi Ito","doi":"10.1111/ncn3.12810","DOIUrl":"https://doi.org/10.1111/ncn3.12810","url":null,"abstract":"Although intravenous methylprednisolone pulse (IVMP) therapy has been recommended for ocular myasthenia gravis (OMG), the evidence is limited.We aimed to investigate the efficacy and safety of IVMP for OMG.The patients with OMG were chosen retrospectively from May 2010 to December 2022. The therapeutic effects between IVMP‐treated and non‐IVMP‐treated groups were determined by ∆ocular quantitative MG (QMG) score and the ∆ocular MG activities of daily living profile (MG‐ADL) score, which is the disparities of the scores between before and after 1 month of drug administration; and Myasthenia Gravis Foundation of America post‐intervention status at 1, 3, 6, and 12 months.There were 26 patients with OMG included, with the mean age of 67.2 ± 13.1 years. 13 of the 26 people with OMG received IVMP and subsequent low‐dose immunotherapies. The ∆ocular QMG and ∆ocular MG‐ADL scores were significantly higher in the IVMP group of 3 (1.5, 4) and 3 (2, 4.5) than in the non‐IVMP group of 1.5 (0, 3) and 1 (0, 2), p = 0.038 and p = 0.0027, respectively. The rates of minimal manifestation or better status in post‐intervention status at 1 and 12 months were also considerably higher in the IVMP group of 77% and 92% compared with the non‐IVMP group of 0% and 23%, p = 0.0001 and 0.001. There were no serious side effects discovered.IVMP induction therapy can quickly and safely improve symptoms of OMG, and subsequent low‐dose immunotherapy can keep symptoms at bay for 12 months.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140365862","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":"Pseudohyperkalemia in myotonic dystrophy type 1: A case report","authors":"T. Hatano, Toyoyoshi Uchida, Hidemori Hayashi, N. Hattori","doi":"10.1111/ncn3.12812","DOIUrl":"https://doi.org/10.1111/ncn3.12812","url":null,"abstract":"Myotonic dystrophy type 1 (DM1) is an autosomal dominant familial muscular dystrophy caused by abnormal CTG repeat expansion in the myotonic dystrophy protein kinase (DMPK) gene. The cardinal features of DM1 patients are muscular weakness, myotonia, and arrhythmia. DM1 patients with electrolyte imbalance caused by endocrinological alterations have also been reported. Herein, we report a female patient with DM1 and hyperkalemia, which fluctuated depending on the blood collection methods. We revealed that cold stimulation of red blood cells was associated with hyperkalemia, whereas blood examination immediately after collection showed normal potassium levels. She was, therefore, diagnosed with pseudohyperkalemia. Several previous reports have described DM1 patients with pseudohyperkalemia, similar to ours. Neurologists should be aware that some patients with DM1 may have pseudohyperkalemia. Thus, consider retesting with prompt measurement after blood collection to rule out pseudohyperkalemia when a patient shows hyperkalemia. If confirmed, evaluate for DM1 as a potential differential diagnosis.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140369966","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":"Optic neuritis as an immune‐related adverse event of pembrolizumab","authors":"Isana Nakajima, Tomoko Nakaizumi, Hideki Tsuji","doi":"10.1111/ncn3.12811","DOIUrl":"https://doi.org/10.1111/ncn3.12811","url":null,"abstract":"","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140372702","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}