Clinical Neurology最新文献

{"title":"[Insular lobe epilepsy. Part 2: presurgical evaluation & surgical interventions with stereo-electroencephalography].","authors":"Koichi Hagiwara","doi":"10.5692/clinicalneurol.cn-001930-2","DOIUrl":"10.5692/clinicalneurol.cn-001930-2","url":null,"abstract":"<p><p>Identification of insular lobe epilepsy (ILE) presents a major clinical challenge in the diagnosis and treatment of drug-resistant focal epilepsies. ILE has diverse clinical presentations due to the multifaceted functions of the insula. Surface EEG findings do not provide straightforward information to predict this deeply-situated origin of seizures; they are even misleading, masquerading as those of other focal epilepsies, such as temporal and frontal ones. Non-invasive imagings may disclose insular abnormalities, but extra-insular abnormalities can coexist or even stand out. Careful reading and a second-look guided by other clinical information are crucial in order not to miss subtle insulo-opercular abnormalities. Furthermore, a possible insular origin of seizures should be considered in MRI-negative frontal/temporal/parietal epilepsies. Therefore, exploration/exclusion of insular-origin seizures is necessary for a great majority of surgical candidates. As for the stereo-electroencephalography, considered as the gold standard method for intra-cranial EEG investigations with suspicion of ILE, planning of electrode positions/trajectories require sufficient knowledge of the functional localization and anatomo-functional connectivity of the insula. Dense sampling within the insula is required in patients with probable ILE, because the seizure-onset zone can be restricted to a single insular gyrus or even a part of it. It is also crucial to explore extra-insular regions on the basis of non-invasive investigation results while considering their anatomo-functional relationships with the insula. From a surgical perspective, differentiating seizures strictly confined to the insula from those extending to the opercula is of particular importance. Pure insular seizures can be treated with less invasive measures, such as radiofrequency thermocoagulation. To conclude, close attention must be paid to the possibility of ILE throughout the diagnostic workup. The precise identification/exclusion of ILE is a prerequisite to provide appropriate and effective surgical treatment in pharmaco-resistant focal epilepsies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"540-549"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789398","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":"[Two patients of immunotherapy-responsive autoimmune cerebellar ataxia fulfilled with criteria of multiple system atrophy].","authors":"Kazuhiro Higashida, Yoya Ono, Masahiko Kato, Akira Takekoshi, Nobuaki Yoshikura, Akio Kimura, Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-001979","DOIUrl":"10.5692/clinicalneurol.cn-001979","url":null,"abstract":"<p><p>We report two patients with autoimmune cerebellar ataxia who fulfilled the diagnostic criteria of multiple system atrophy (MSA) and responded to immunotherapies. Patient 1 was a 72-year-old man who was diagnosed with clinically probable MSA according to Movement Disorder Society criteria. Patient 2 was a 68-year-old man who was diagnosed with clinically established MSA according to Movement Disorder Society criteria. Both patients showed cerebellar ataxia, autonomic dysfunction, and pyramidal tract signs; however, they also had atypical clinical features. Patient 1 exhibited self-‍limiting mild improvement of clinical symptoms and had inflammatory findings in his cerebrospinal fluid. Patient 2 showed a rapidly progressive clinical course. We therefore examined anti-neuronal antibodies using tissue-based immunohistochemical assays with frozen rat cerebellum sections. We detected autoantibodies that mainly reacted with the cytoplasm of Purkinje cells. The two patients then underwent immunotherapies, which led to substantial improvements in their clinical symptoms. Our findings indicate that some patients with autoimmune cerebella ataxia have clinical features that resemble MSA, and respond well to immunotherapies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"589-593"},"PeriodicalIF":0.0,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141761546","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":"[Abnormal nocturnal behavior mimicking REM sleep behavior disorder episodes in a patient with periodic limb movement disorder].","authors":"Kaoru Senzaki, Mutsumi Okura, Yoshinobu Ohnishi","doi":"10.5692/clinicalneurol.cn-001986","DOIUrl":"10.5692/clinicalneurol.cn-001986","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"498-499"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443478","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":"[Malignant transformation of Ollier disease-related multiple glioma with IDH1 p.R132C mutation].","authors":"Gen Watanabe, Yu Fujii, Yoshiki Hanaoka, Miyuki Tanaka, Mai Iwaya, Tetsuyoshi Horiuchi","doi":"10.5692/clinicalneurol.cn-001955","DOIUrl":"10.5692/clinicalneurol.cn-001955","url":null,"abstract":"<p><p>A 21-year-old man who was diagnosed with Ollier disease at the age of 1 year developed incidental multiple gliomas at the age of 15 years. Subsequently, the multiple gliomas enlarged and the patient underwent three surgical removals. Genetic analysis revealed the IDH1 p.R132C mutation in the gliomas, and histopathology showed malignant transformation. Despite multimodality treatment, the gliomas could not be controlled, and the patient died at the age of 23 years. Ollier disease is a rare disease with IDH1/2 mutations and is often associated with gliomas. However, there are very few reports on genetic analysis of IDH1/2 mutations and long-term follow-up in Ollier disease-related gliomas. Genetic analysis of IDH mutations may contribute to the elucidation of its pathogenesis. The cross-departmental collaboration is required for long-term follow-up of Ollier disease-related gliomas.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"474-479"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141427831","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":"[Issues of transition support to adulthood and the practices of pediatric-adult healthcare transition in neurological diseases].","authors":"Katsuhisa Ogata, Yoko Mochizuki, Satoko Kumada, Sunao Tomita, Yoshio Sakiyama, Kenjiro Kikuchi, Mika Hayakawa, Miho Osako, Toshio Saito, Hideki Mochizuki","doi":"10.5692/clinicalneurol.cn-001985","DOIUrl":"10.5692/clinicalneurol.cn-001985","url":null,"abstract":"<p><p>A workshop of the Special Committee on Measures for Transition from Pediatric to Adult Health Care, the Japanese Society of Neurology was held to discuss various issues and practices involved in healthcare transition. The following points were addressed: (1) the history of, and issues involved in, promoting support for patients requiring medical care, (2) cooperation between pediatric medical centers and university hospitals, (3) collaboration between pediatrics and neurology in medical and rehabilitation facilities, and (4) a questionnaire survey of members of the Japanese Society of Neurology. The reasons for extreme difficulties in pediatric-adult healthcare transition for patients with neurological diseases, especially those who require continuous intensive medical care over a long period of time, include the difference in the operating systems of pediatric and adult departments, in addition to the difference in the diseases treated during childhood and adulthood. For holistic transition support, it is necessary to strengthen cooperation not only among medical professionals, but also among multiple professions, as well as between local communities and government.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"460-464"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443480","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":"[Diagnosis of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis led by sarcoplasmic myxovirus resistance protein A expression on muscle pathology].","authors":"Kosuke Iwami, Takahiro Kano, Keiichi Mizushima, Hiroaki Yaguchi, Ichizo Nishino, Hideki Houzen","doi":"10.5692/clinicalneurol.cn-001963","DOIUrl":"10.5692/clinicalneurol.cn-001963","url":null,"abstract":"<p><p>A 44-year-old woman with autism spectrum disorder developed bulbar symptoms and generalized muscle weakness 7 months before referral. Six months before, she was administered glucocorticoid for liver involvement. During the course, while she presented alopecia, skin ulcers, and poikiloderma, hyperCKemia was observed only twice. Due to complications including cardiac involvement and hearing loss as well, we suspected mitochondrial disease and performed a muscle biopsy. The muscle pathology showed sarcoplasmic myxovirus resistance A (MxA) expression with scattered pattern. Since anti-melanoma differentiation-associated gene 5 (MDA5) antibody was detected, we diagnosed the patient with anti-MDA5 antibody-positive dermatomyositis (DM). We reinforced immunosuppressive therapy, and her clinical symptoms and liver involvement were improved. When we diagnose a case of anti-MDA5 antibody-positive DM who is difficult to make clinical diagnosis, it may be valuable to evaluate sarcoplasmic MxA expression on muscle pathology.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"480-485"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141427830","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 recurrent Campylobacter fetus meningitis, occurring two months after the ‍initial infection was successfully treated].","authors":"Satoki Ito, Takuya Tamura, Yuji Ishihara, Haruka Ito, Tomoko Noda, Hiroki Ito","doi":"10.5692/clinicalneurol.cn-001978","DOIUrl":"10.5692/clinicalneurol.cn-001978","url":null,"abstract":"<p><p>A 43-year-old man was admitted to our department due to fever and headache. The cerebrospinal fluid analysis confirmed bacterial meningitis. Campylobacter species were isolated from blood cultures on the third day of admission. The patient was treated with meropenem (MEPM) and discharged on the 17th day. However, he experienced a recurrence of meningitis and was readmitted on the 68th day, initiating MEPM therapy. Campylobacter fetus was isolated from cerebrospinal fluid cultures on the 74th day. MEPM was continued until the 81st day, followed by one month of minocycline (MINO) therapy. The patient had an uneventful recovery without further recurrence. This case highlights the potential for recurrence of Campylobacter fetus meningitis approximately two months after the resolution of the initial infection. In addition to carbapenem therapy for at least two weeks, the adjunctive administration of MINO may be beneficial.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"490-495"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443477","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":"[Brain iron deficiency and periodic limb movement disorder with parasomnias].","authors":"Tomoyuki Miyamoto, Masayuki Miyamoto","doi":"10.5692/clinicalneurol.cn-001962","DOIUrl":"10.5692/clinicalneurol.cn-001962","url":null,"abstract":"","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"496-497"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443479","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":"[Memory impairments in temporal lobe epilepsy].","authors":"Takahiko Mukaino","doi":"10.5692/clinicalneurol.cn-001886","DOIUrl":"10.5692/clinicalneurol.cn-001886","url":null,"abstract":"<p><p>Temporal lobe epilepsy is known to present with various cognitive impairments, among which memory deficits are frequently reported by patients. Memory deficits can be classified into two types: classical hippocampal amnesia, which is characterized by abnormalities detected in neuropsychological assessments, and atypical memory deficits, such as accelerated long-term amnesia and autobiographical memory impairment, which cannot be identified using standard testing methods. These deficits are believed to arise from a complex interplay among structural brain abnormalities, interictal epileptic discharges, pharmacological factors, and psychological states. While fundamental treatments are limited, there are opportunities for interventions such as environmental adjustments and rehabilitation. This review article aims to provide a comprehensive overview of the types, underlying pathophysiology, and intervention methods for memory disorders observed in patients with temporal lobe epilepsy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"453-459"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443481","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 perception gap on migraine: a survey study of the migraine patients, family members, and physicians].","authors":"Shiho Suzuki, Mamoru Shibata, Daisuke Danno, Yoshinori Tanizawa, Satoshi Osaga, Masayuki Hamakawa, Mika Komori","doi":"10.5692/clinicalneurol.cn-001950","DOIUrl":"10.5692/clinicalneurol.cn-001950","url":null,"abstract":"<p><p>Migraine is a disease that is difficult to be recognized by those around the patients, even though it causes significant hindrances. In this study, we conducted an exploratory comparison of the perceptions on migraine among patients, family members living with them, and physicians treating migraine patients. Patients and family members shared a common understanding on the pain of migraine, and hoped to spend more/better time together as a family. However, although family members felt compassion for the patients, lack of understanding by and patients' concern for the surroundings led to feelings of resignation and endurance on the side of patients. Regarding physicians' medical care, our results suggested the importance to understand the wishes and obstacles of each patient and to propose treatment accordingly. In order to reduce the burden of migraine, it is necessary to create an environment and raise awareness that allows people around the patients to understand and support the pain and hopes that each patient feels.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"465-473"},"PeriodicalIF":0.0,"publicationDate":"2024-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141443482","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}