{"title":"Elucidation of the role of microglia‐macrophage‐based neuroinflammation in neurological diseases","authors":"Ryo Yamasaki","doi":"10.1111/ncn3.12829","DOIUrl":"https://doi.org/10.1111/ncn3.12829","url":null,"abstract":"To elucidate the roles of microglia and macrophages in neuroinflammation and their dual impact on the progression of neurological diseases.Microglia and macrophages are integral components of the central and peripheral nervous systems, where they play important roles in maintaining homeostasis, influencing disease progression, and facilitating repair mechanisms.The review integrates recent advancements in single‐cell RNA sequencing that highlight the extensive heterogeneity of microglia and macrophages, which helps in understanding their wide‐ranging roles in different neurological conditions.The analysis reveals that microglia and macrophages have a dual nature, capable of both exacerbating and mitigating disease processes across various conditions, including Alzheimer’s disease, multiple sclerosis, stroke, peripheral nerve disorders, and brain tumors.Modulating the activity of microglia and macrophages offers a promising avenue for therapeutic interventions in neurological disorders. There is a critical need for further research to fully leverage their therapeutic potential in targeting neuroinflammation to enhance patient outcomes.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141108156","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}
Vedang Desai, J. Parihar, A. Elavarasi, Animesh Das, R. Singh, D. Vibha, Shailesh B. Gaikwad, Manjari Tripathi
{"title":"Drug‐refractory epilepsy as presenting feature of Niemann–Pick disease","authors":"Vedang Desai, J. Parihar, A. Elavarasi, Animesh Das, R. Singh, D. Vibha, Shailesh B. Gaikwad, Manjari Tripathi","doi":"10.1111/ncn3.12833","DOIUrl":"https://doi.org/10.1111/ncn3.12833","url":null,"abstract":"Niemann–Pick disease type C (NPC) is a lipid storage disorder having varied neurological, psychiatric, and visceral manifestations. Twenty‐seven‐year‐old female presented to the hospital with recurrent episodes of seizures for the last one and half year. She had delayed motor and language milestones and was scholastically poor in school. On examination, vertical saccades were slow with downgaze restriction. Higher mental function examination revealed predominant impairment of frontal and temporal lobe functions. She also had intermittent oromandibular dyskinesis. Cerebrospinal fluid analysis showed nil cells with protein 28 mg/dL and glucose 54 mg/dL. MRI brain showed diffuse supratentorial cerebral atrophy with atrophy of corpus callosum and multiple subcortical and periventricular T2/FLAIR hyperintensities. A clinical suspicion of NPC was considered and genetic analysis showed homozygous mutation of the NPC‐2 gene on Exon 3. So, our case was unique in terms of drug‐refractory epilepsy was the presenting feature that led to the diagnosis of NPC.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141115542","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}
Naoki Yamahara, N. Yoshikura, Akio Kimura, Mayu Sakai, Daisuke Yabe, T. Shimohata
{"title":"Obsessive‐compulsive disorder as an initial manifestation of anti‐glutamic acid decarboxylase antibody‐associated encephalitis","authors":"Naoki Yamahara, N. Yoshikura, Akio Kimura, Mayu Sakai, Daisuke Yabe, T. Shimohata","doi":"10.1111/ncn3.12830","DOIUrl":"https://doi.org/10.1111/ncn3.12830","url":null,"abstract":"We present the case of a 44‐year‐old woman with type 1 diabetes and anti‐glutamic acid decarboxylase antibody (GAD‐Ab) (6100 U/mL in serum), manifesting as a one‐month history of compulsive writing in her self‐monitoring blood glucose (SMBG) diary and a one‐week history of seizures before admission. On admission, she was diagnosed with GAD‐Ab‐associated encephalitis (GAD‐AE), and compulsive writing consistent with obsessive‐compulsive disorder (OCD) caused by a repetitive and persistent urge to neutralize anxiety manifesting as clearly excessive writing. Immunotherapy, including corticosteroids, azathioprine, and intravenous immunoglobulin, improved her compulsive writing, and seizures disappeared following lacosamide treatment. Two months after discharge, compulsive writing reappeared and the patient was successfully treated with temporary immunotherapy intensification. After a stable period of 2 years, the patient died due to hypoglycemic encephalopathy. Overall, we showed that GAD‐AE may cause OCD.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141126456","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}
Honami Kawai, Y. Nishida, Taro Ishiguro, M. Akaza, Shinsuke Miyazaki, Takanori Yokota
{"title":"Severe respiratory failure secondary to unilateral phrenic nerve injury: A case report","authors":"Honami Kawai, Y. Nishida, Taro Ishiguro, M. Akaza, Shinsuke Miyazaki, Takanori Yokota","doi":"10.1111/ncn3.12832","DOIUrl":"https://doi.org/10.1111/ncn3.12832","url":null,"abstract":"Patients with unilateral phrenic nerve paralysis are usually asymptomatic at rest. We present a 77‐year‐old woman with a chest wall osteoarthropathy associated with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome that developed severe hypercapnic respiratory failure following cryoballoon ablation for the management of atrial fibrillation. Her right phrenic nerve injury (PNI) was confirmed by chest radiography, phrenic nerve conduction studies, and diaphragmatic ultrasonography. We conclude that unilateral PNI during ablation invalidated the diaphragmatic compensation for pre‐existing reduced thoracic expansion, resulted in severe hypoventilation. Clinicians should be careful of the fact even unilateral PNI could cause catastrophic respiratory failure in patients with restricted thoracic expansion.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140963087","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}
Hiroyasu Inoue, M. Oomura, Y. Madokoro, Yoko Taniguchi, Kengo Suzuki, Toyohiro Sato, T. Fujioka, Masayuki Mizuno, S. Kawashima, K. Okita, Kenichi Yoshimura, Noriyuki Matsukawa
{"title":"Efficacy of prasugrel versus clopidogrel for early neurological deterioration in branch atheromatous disease: An exploratory study","authors":"Hiroyasu Inoue, M. Oomura, Y. Madokoro, Yoko Taniguchi, Kengo Suzuki, Toyohiro Sato, T. Fujioka, Masayuki Mizuno, S. Kawashima, K. Okita, Kenichi Yoshimura, Noriyuki Matsukawa","doi":"10.1111/ncn3.12828","DOIUrl":"https://doi.org/10.1111/ncn3.12828","url":null,"abstract":"Dual antiplatelet therapy (DAPT) with clopidogrel is one of the initial treatment regimens for early neurological deterioration (END) in branch atheromatous disease (BAD). However, its effectiveness is affected by cytochrome P450 2C19 polymorphism. Prasugrel, with reduced cytochrome P450 2C19 interactions, has been approved for cerebrovascular diseases in Japan.To explore the efficacy of DAPT with prasugrel and with clopidogrel in preventing END.Patients with BAD, admitted within 48 h of symptom onset between June 2022 and September 2023, were enrolled. The primary endpoint was the proportion of patients with END within 7 days, which was defined as an increase of 1 point or more on the National Institutes of Health Stroke Scale (NIHSS). Patients meeting specific magnetic resonance imaging (MRI) criteria, including lesions of ≥15 mm in the lenticulostriate artery (LSA) region or infarcts extending to the ventral side of the paramedian pontine region, were included. The patients were randomly assigned to the clopidogrel with loading and prasugrel without loading groups and administered common medications.The study included 9 and 10 patients in clopidogrel and prasugrel groups, of whom 56% and 50% had END, respectively. The prasugrel group had earlier exacerbations after admission (days 0–1) than that of the clopidogrel group (days 1–2). The bleeding complications of both groups were not significantly different.The two groups had similar proportions of patients with END, suggesting comparable efficacies of both drugs for patients with BAD.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140995814","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}
Yasuko Mori, S. Miyatake, K. Kunieda, N. Yoshikura, Yuichi Hayashi, K. Higashida, Akio Kimura, E. Koshimizu, Naomichi Matsumoto, T. Shimohata
{"title":"A cerebellar ataxia patient harboring 229 pure GAA repeat units in FGF14 presenting with grip myotonia","authors":"Yasuko Mori, S. Miyatake, K. Kunieda, N. Yoshikura, Yuichi Hayashi, K. Higashida, Akio Kimura, E. Koshimizu, Naomichi Matsumoto, T. Shimohata","doi":"10.1111/ncn3.12826","DOIUrl":"https://doi.org/10.1111/ncn3.12826","url":null,"abstract":"Spinocerebellar ataxia 27 B (SCA27B) is caused by the expansion of GAA repeats in the intron of the fibroblast growth factor 14 (FGF14) on chromosome 13 and is inherited dominantly. An 80‐year‐old male visited the hospital complaining of ataxic gait and harboring 229 pure GAA repeat units in the FGF14. Almost all the clinical features were similar to that of SCA27B. However, the patient initially presented with episodic grip myotonia, which has not been previously reported.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141006372","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}
S. Tetsuka, Jun Sawada, Tomohiro Suzuki, Tomoko Ogawa, R. Hashimoto
{"title":"Diagnostic value of T2*‐weighted magnetic resonance imaging in hematomyelia","authors":"S. Tetsuka, Jun Sawada, Tomohiro Suzuki, Tomoko Ogawa, R. Hashimoto","doi":"10.1111/ncn3.12823","DOIUrl":"https://doi.org/10.1111/ncn3.12823","url":null,"abstract":"","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140691547","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}
Akinori Kinoshita, Kota Sato, Yoshimi Shinohara, T. Ohtonari, Yuka Terasawa, Yutaka Shimoe, Tatsuo Kohriyama
{"title":"A case of thoracic epidural angiolipoma with intracranial hypertension","authors":"Akinori Kinoshita, Kota Sato, Yoshimi Shinohara, T. Ohtonari, Yuka Terasawa, Yutaka Shimoe, Tatsuo Kohriyama","doi":"10.1111/ncn3.12816","DOIUrl":"https://doi.org/10.1111/ncn3.12816","url":null,"abstract":"Spinal cord tumors sometimes present with intracranial hypertension and can be difficult to diagnose at the initial presentation without symptoms due to spinal cord compression. Here, we report a case of a patient with thoracic epidural angiolipoma complicated by increased intracranial pressure and normal protein levels in cerebrospinal fluid (CSF). His neurological symptoms improved immediately after partial removal of the core part of epidural angiolipoma. Spinal cord lesions should be ruled out in cases with intracranial hypertension, even if the CSF protein levels are normal.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140696752","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":"Istradefylline/L‐DOPA Parkinson's disease therapy and energy coupling","authors":"Naomi Kanzato, Koh Nakachi, Satsuki Mochizuki","doi":"10.1111/ncn3.12820","DOIUrl":"https://doi.org/10.1111/ncn3.12820","url":null,"abstract":"Both dopamine and adenosine are physiologically involved in cellular energy metabolism.Novel approaches targeting delaying progression strategies for Parkinson's disease (PD) examined the effects of long‐term use of A2AR antagonist/istradefylline (IST) adjunct to L‐DOPA (LD), nutshell IST‐LD, with an expected energy coupling (EC) to attenuate neuronal excitotoxicity.A single‐center prospective open cohort study was conducted at the Department of Neurology, Okinawa Islands, Japan. The cohort included incident and prevalent PD cases that were non‐randomly assigned to the IST‐LD (n = 90) and LD (n = 157) groups, followed by an open‐label treatment in clinical practice from October 2013 to September 2020. Patients were evaluated using physician‐rated MDS‐UPDRS I/III, and patient‐rated MDS‐NMS using the total score, body weight, and blood biomarkers of EC.As the primary clinical outcome, MDS‐UPDRS part III scores showed less worsening in the IST‐LD than in the LD group (1.29 ± 6.0 vs. 5.0 ± 6.5, p < 0.01) and off‐time Hoehn and Yahr stage (0.05 ± 0.49 vs. 0.26 ± 0.54, p < 0.01). As the secondary clinical outcome, the detected mean body weight losses were similar between the two groups; however, the evident linkage of the daily dose of LD and variations in the blood markers of IGF‐I and HKII, which were inferred as LD‐mediated energy demand effects, were partially attenuated by IST.IST‐LD plays an essential role in behavioral outputs for patients with PD and might contribute to the interplay of energy‐consuming demands of LD with the attenuation of weight loss and emerging type 2 diabetes mellitus.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140702490","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":"Management of multiple cerebral embolisms due to polycythemia vera with acquired von Willebrand syndrome","authors":"Masashi Watanabe, Haruki Ohtsubo, Akira Shiraoka, Itaru Kyoraku, Kensho Okamoto","doi":"10.1111/ncn3.12822","DOIUrl":"https://doi.org/10.1111/ncn3.12822","url":null,"abstract":"Polycythemia vera (PV) is a rare blood disorder associated with thrombosis or acquired von Willebrand syndrome (AvWS); however, their coexistence is unusual. Here, we report about a 59‐year‐old man with PV who developed both ischemic stroke (IS) and AvWS. Although the patient had no history of thrombosis, he had refractory epistaxis 2 years prior. Brain magnetic resonance imaging and angiography (MRI and MRA) revealed new IS and mural thrombi at the left distal carotid artery. Laboratory investigations showed elevated hemoglobin and hematocrit levels with mild leukocytosis and thrombocytosis, corresponding to PV. Additionally, suppressed platelet aggregation and activity despite normal antigen of von Willebrand factor indicated AvWS. Appropriate repeated phlebotomies successfully resolved the MRA abnormalities and prevented IS recurrence, although the patient refused the recommended treatment with hydroxyurea. This case highlights the need for careful management to prevent recurrent IS and major bleeding in patients with PV who develop IS accompanying AvWS.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140700433","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}