Brain disorders (Amsterdam, Netherlands)最新文献

{"title":"Silent struggles: Mapping the terrain of sleep, anxiety, and depression in the Era of pandemic for individuals with multiple sclerosis- a cross-sectional study","authors":"Bindu Balakrishnan ,&nbsp;Vijay Prakash Krishnan Muthaiah ,&nbsp;Miguel Garcia ,&nbsp;Hina Garg ,&nbsp;Mohan Ganesan","doi":"10.1016/j.dscb.2024.100149","DOIUrl":"https://doi.org/10.1016/j.dscb.2024.100149","url":null,"abstract":"<div><h3>Background</h3><p>The pandemic's preventive measures adversely impacted physical and psychological well-being, leading to increased sleep issues and mental health problems. This study examines sleep health and mental well-being among individuals with multiple sclerosis (MS) during this period. It explores potential correlations between sleep problems and mental health in this population.</p></div><div><h3>Methods</h3><p>A cross-sectional design utilized an anonymous SurveyMonkey survey distributed to three MS centers, collecting responses from 24 individuals with MS (21 females, 3 males). Sleep data were assessed using the Functional Outcome of Sleep Questionnaire (FSOQ)-10, and mental health status was assessed using the Depression Anxiety Stress Scale (DASS)-21. FOSQ scores ≥17.9 indicated normal sleep, while the DASS-21 comprised stress, anxiety, and depression subscales, each with 7 questions rated on a 0–3 scale, with higher scores indicating greater mental health issues.</p></div><div><h3>Results</h3><p>Of 24 participants, 18 MS individuals reported functional sleep issues during the pandemic. Analysis revealed significantly lower productivity, vigilance, and social outcomes in the sleep problems group (<em>p</em> &lt; .01). Additionally, this group had elevated stress (<em>p</em> = .004) and depression scores (<em>p</em> = .002). Negative correlations were observed between sleep scores and both stress (ρ = -0.485, <em>p</em> = .016) and depression (ρ = -0.562, <em>p</em> = .004).</p></div><div><h3>Conclusions</h3><p>75 % of MS individuals experienced functional sleep disturbances during the pandemic, with 25 % affected by stress, 16.6 % by anxiety, and 37 % by depression. Sleep issues are correlated with increased stress and depression levels. Recognizing the impact on rehabilitative outcomes, specialists must consider sleep behavior and mental health when designing therapies for individuals with MS.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"15 ","pages":"Article 100149"},"PeriodicalIF":0.0,"publicationDate":"2024-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000349/pdfft?md5=25e7e47b316a153df4ca0133f87a009c&pid=1-s2.0-S2666459324000349-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141540068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burr-hole assisted direct transcranial Onyx embolization of an isolated transverse sinus dural arteriovenous fistula","authors":"Arevik Abramyan ,&nbsp;Gaurav Gupta ,&nbsp;Emad Nourollah-Zadeh ,&nbsp;Srihari Sundararajan ,&nbsp;Sudipta Roychowdhury","doi":"10.1016/j.dscb.2024.100147","DOIUrl":"https://doi.org/10.1016/j.dscb.2024.100147","url":null,"abstract":"<div><h3>Introduction</h3><p>Treating dural arteriovenous fistulas (DAVFs) with endovascular embolization has proven to be effective. However, in cases where the DAVF is isolated or inaccessible via conventional endovascular methods, a combined surgical and endovascular approach may be necessary. This strategy provides a direct approach to the venous system and bypasses the site of sinus occlusion.</p></div><div><h3>Methods</h3><p>In this report, we present a case of direct Onyx embolization after navigated cannulation of the transverse sinus (TS) for the treatment of a left TS DAVF, along with a review of the literature on transcranial endovascular treatment of DAVF.</p></div><div><h3>Results</h3><p>The isolated TS was embolized with Onyx, filling both supratentorial and infratentorial draining cortical varices. The patient tolerated the procedure well, and follow-up angiography revealed no DAVF recurrence.</p></div><div><h3>Discussion</h3><p>Onyx has been used in very few case reports for transcranial DAVF embolization, even though its higher viscosity characteristics offer better control over distal migration at the possible cost of decreased penetration of the targeted lesion. When using coils, the sinus exposure must be long enough to allow for puncture at a certain distance from the DAVF. The superficial location of the isolated TS allowed us to use a syringe filled with Onyx to directly obliterate the sinus during a single burr-hole craniotomy.</p></div><div><h3>Conclusions</h3><p>This combined strategy might be a practical and efficient way to treat DAVFs with sinus occlusion. It can serve as the primary treatment option when addressing a DAVF involving a superficial sinus with drainage disconnected from both internal jugular veins.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"15 ","pages":"Article 100147"},"PeriodicalIF":0.0,"publicationDate":"2024-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000325/pdfft?md5=9a7ed80581570992655d83c48d2c863e&pid=1-s2.0-S2666459324000325-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141481290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chatbots in neurology and neuroscience: Interactions with students, patients and neurologists","authors":"Stefano Sandrone","doi":"10.1016/j.dscb.2024.100145","DOIUrl":"10.1016/j.dscb.2024.100145","url":null,"abstract":"<div><p>Chatbots, also known as conversational agents, can guide customers in navigating websites, answer questions or act as personal assistants. They have recently entered the neuroscientific arena and can potentially revolutionise neurology and neuroscience. In this manuscript, we explore what chatbots can and cannot do in their interactions with students, patients and neurologists, and we analyse their potentialities and limitations.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"15 ","pages":"Article 100145"},"PeriodicalIF":0.0,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000301/pdfft?md5=ad4932b9d500b268d426f91e05317081&pid=1-s2.0-S2666459324000301-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141407826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-transgenic models of Parkinson's disease: The pursuit of a resemblance","authors":"Letícia Germino Veras,&nbsp;Henrique de Oliveira Amaral,&nbsp;Isabela Ferreira Lima Mota,&nbsp;Caio Vinícius Ibias Belardinelli de Azevedo,&nbsp;Yasmin Costa e Carrilho,&nbsp;Márcia Renata Mortari,&nbsp;Bruna Lotufo Denucci","doi":"10.1016/j.dscb.2024.100143","DOIUrl":"10.1016/j.dscb.2024.100143","url":null,"abstract":"<div><p>Parkinson's disease is the second most frequent neurodegenerative disorder worldwide and is characterized by the presence of predominantly motor symptoms, the loss of dopaminergic neurons, and degeneration of the striatum. In addition to the motor symptoms of Parkinson´s disease, there are other non-motor symptoms such as hyposmia, sleep disorders, personality changes, pain, depression, gait, speech, and swallowing difficulties. Although there has been much research in the field, there is still no specific treatment for the disease. Current studies for new treatments use a variety of models, and the most used animals are rodents, primates, and non-mammals. This review aims to present the non-transgenic models for Parkinson's disease, providing an overview of each model, their advantages and disadvantages, and at last, how they could be helpful in the search for new treatments.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100143"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000283/pdfft?md5=1b359d0fcfea6ccf93c7f3374604d25a&pid=1-s2.0-S2666459324000283-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141133934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Morphometric evaluation of the foramen magnum in the West African population: Implications for neurosurgical interventions","authors":"D.E. Ogolo ,&nbsp;E.C. Ajare ,&nbsp;O. Okwuoma ,&nbsp;K.O. Ndukuba ,&nbsp;S. Nnama ,&nbsp;C.A. Ndubuisi ,&nbsp;W.C. Mezue ,&nbsp;S.C. Ohaegbulam","doi":"10.1016/j.dscb.2024.100140","DOIUrl":"https://doi.org/10.1016/j.dscb.2024.100140","url":null,"abstract":"<div><h3>Background and objectives</h3><p>While various pathologies affecting the foramen magnum region can have severe consequences, little research has been conducted on the unique morphological patterns in the West African subregion. The study aimed to assess these patterns and their implications for surgeries, comparing them with global standards.</p></div><div><h3>Methods</h3><p>A descriptive study was conducted on 315 patients over a two-year period, excluding those with specific abnormalities. Measurements obtained from cranial 1.5T MRI scans included anteroposterior and transverse diameters of the foramen magnum. From these, the foramen magnum area and index were calculated. The data was analyzed by inferential, comparative and descriptive statistics, and a <em>p</em> value &lt; 0.05 was regarded as statistically significant.</p></div><div><h3>Results</h3><p>On average, the transverse and anteroposterior diameters were 28.51 mm and 33.02 mm for males and 28.39 mm and 33.47 mm for females, with a slightly smaller foramen magnum area in males (7.42 cm²) compared to females (7.47 cm²). Despite these differences, the variations were not statistically significant. However, the foramen magnum indices indicated medium size configuration for females and large size configuration for males, aligning with global trends.</p></div><div><h3>Conclusion</h3><p>The study concluded that West Africans exhibited lower foramen magnum area and indices compared to other regions, with minor differences between sexes. Females tended to have a medium size configuration, while males tended to have a larger size configuration. These findings provide valuable insights for clinicians, highlighting the importance of considering ethno-regional variations in surgical approaches and interventions related to the craniocervical junction.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100140"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000258/pdfft?md5=d1c0f2bff77dcae4a1590ccc886660a0&pid=1-s2.0-S2666459324000258-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141242813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of anesthesia in endovascular therapy management of ischemic stroke: A systematic review and meta-analysis","authors":"Thuraya N. Al-Sayegh ,&nbsp;Bayan O. Abu Alragheb ,&nbsp;Moayad N. Aldahabi ,&nbsp;Ahmad A. Toubasi","doi":"10.1016/j.dscb.2024.100144","DOIUrl":"10.1016/j.dscb.2024.100144","url":null,"abstract":"<div><h3>Background</h3><p>Endovascular treatment and thrombolytic therapy are widely used as the standard treatment for acute ischemic strokes. General Anesthesia (GA) is preferred over Conscious Sedation (CS) because it is associated with lower pain, anxiety, agitation and aspiration risk. However time is very fundemental in acute ischemic stroke treatment.</p></div><div><h3>Methods</h3><p>The search was done on the 30th of December 2022. The inclusion criteria of the studies were if they were randomized controlled clinical trials in design and compared between using conscious sedation and general anesthesia in endovascular treatment along with thrombolytic therapy.</p></div><div><h3>Results</h3><p>The total number of patients included in our analysis was 743 from six clinical trials. Our models showed a significant association between conscious sedation and improved disability (OR=1.32; 95%CI: 1.10–1.59). The model demonstrated that conscious sedation was significantly associated with lower mean stroke to procedure time (WMD=-11.93; 95%CI: -23.72- -0.14) and lower mean hospital arrival to procedure time (WMD=-8.30; 95%CI: -13.88- -2.72). Moreover, our analysis revealed that conscious sedation was significantly associated with lower hypotensive episodes (WMD=0.67; 95%CI: 0.48–0.95) and lower use of vasoactive drugs (WMD=0.75; 95%CI: 0.57–0.99). There were no significant differences between the two study groups in technical failures, successful recanalization or mortality.</p></div><div><h3>Conclusion</h3><p>CS demonstrated improved disability, shorter times to procedure, fewer hypotensive events, and lower use of vasoactive drugs. All of which reflect a better quality for CS over GA. Given together, these findings can help guide the clinical decision which should be tailored to the patients’ needs.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100144"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000295/pdfft?md5=c01505b0152a6b3d4e7df44fe48b8cdf&pid=1-s2.0-S2666459324000295-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141278550","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From location to manifestation: a systematic review and meta-analysis of seizure prevalence in different brain tumor sites","authors":"Christine Audrey ,&nbsp;Kheng-Seang Lim ,&nbsp;Rafdzah Ahmad Zaki ,&nbsp;Vairavan Narayanan ,&nbsp;Si-Lei Fong ,&nbsp;Chong-Tin Tan","doi":"10.1016/j.dscb.2024.100146","DOIUrl":"https://doi.org/10.1016/j.dscb.2024.100146","url":null,"abstract":"<div><h3>Objectives</h3><p>The location of the brain tumor in the frontal or temporal lobes has been reported as a predictor for seizure occurrence in patients with a brain tumor but the predictive value of other locations is uncertain. We aimed to ascertain the pooled prevalence of pre-operative seizures in relation to the brain tumor locations, by employing a systematic review and meta-analysis.</p></div><div><h3>Methods</h3><p>The search was conducted up to 1st May 2023, in Pubmed, Embase, and Web of Science and references were exported and managed using EndNote 20. Articles were included if they reported a prevalence or incidence of the seizure at the tumor location. MetaXL by Epigear was used to generate the meta-analysis.</p></div><div><h3>Results</h3><p>The pooled prevalence for preoperative seizures in gliomas was 51–63% in most locations (frontal, temporal, parietal, and insula) but lowest in the occipital lobe (28%). Subgroup analysis on low-grade gliomas showed a high pre-operative seizure prevalence in all locations: frontal lobe [75% (95% CI: 68–81%)], temporal lobe [73% (95% CI: 63–82%)], parietal lobe [79% (95% CI: 57–82%)], occipital lobe [46% (95% CI: 0–95%)], and insular [76% (95% CI: 65–77%)]. In the astrocytoma and meningioma subanalysis, the pooled prevalence is ≥ 40% in most cortical location. In brain metastases, the pooled prevalence was similar in most locations (18–39%), lowest in the temporal lobe.</p></div><div><h3>Conclusion</h3><p>Tumor location is not the main influencing factor for pre-operative seizure independent from tumor type. Extensive seizure screening should be considered irrespective of tumor location, especially in those with low-grade gliomas.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100146"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000313/pdfft?md5=f955cda00a8cf5bca8ae9f60eb19bb57&pid=1-s2.0-S2666459324000313-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141479481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clozapine abrupt cessation fueling overlap neuroleptic malignant syndrome and serotonin syndrome with oculomotor dysfunction: a case report","authors":"Khaled Zammar ,&nbsp;Majd A․AbuAlrob ,&nbsp;Ahmad Meer ,&nbsp;Boulenouar Mesraoua ,&nbsp;Abeer Safan","doi":"10.1016/j.dscb.2024.100141","DOIUrl":"10.1016/j.dscb.2024.100141","url":null,"abstract":"<div><p>Neuroleptic Malignant Syndrome (NMS) and Serotonin Syndrome (SS) are life-threatening conditions caused by adverse reactions to neuroleptics and antidepressant medications. The overlap of both syndromes is rare but has been described previously. Differentiating between both entities based on the clinical presentation can be difficult. We present a case of a young male patient with history of schizophrenia, admitted to the Medical Intensive Care Unit (MICU) for septic shock, who developed NMS and SS with oculomotor dysfunction following the abrupt cessation of clozapine while still being on an antidepressant.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100141"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S266645932400026X/pdfft?md5=41811a0dde0ec47743009fd1dadc3147&pid=1-s2.0-S266645932400026X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141141760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inhibitory influence of agmatine on catamenial-like seizure susceptibility in progesterone withdrawn female rats","authors":"Madhura Dixit Vinchurney ,&nbsp;Vaishali Gandhare ,&nbsp;Milind Umekar ,&nbsp;Nazma Inamdar ,&nbsp;Brijesh Taksande ,&nbsp;Nandkishor Kotagale","doi":"10.1016/j.dscb.2024.100142","DOIUrl":"10.1016/j.dscb.2024.100142","url":null,"abstract":"<div><p>Catamenial epilepsy is a condition marked by an increased occurrence of seizures that coincide with the menstrual cycle in women. Our study explored the role of neurotransmitter/neuromodulator agmatine in progesterone withdrawal induced increased seizure susceptibility that is one of the causes of catamenial epilepsy. Additionally, it investigates potential interactions between agmatine and the central neurosteroid system. The experiments involved the use of female Sprague-Dawley rats. To mime the seizure susceptibility as seen in catamenial epilepsy, rats were treated with PMSG and b-HCG which cause a higher release of progesterone and later injected with Finasteride on the 11th day of post-human chorionic gonadotropin administration to cause a sudden drop of progesterone. On the 12th day, seizure susceptibility was assessed through a low-dose (35 mg/kg) pentylenetetrazol (PTZ) injection. The results revealed that agmatine treatment showed a protective effect against seizures. Furthermore, this study explored the impact of neurosteroids such as allopregnanolone or progesterone (a precursor of allopregnanolone) and neurosteroidogenic drugs like FGIN 1–27 or metyrapone (an 11β-hydroxylase inhibitor) on the anticonvulsant effect of agmatine. The findings indicated that prior administration of these substances significantly enhanced the anticonvulsant effect of agmatine. In contrast, inhibiting neurosteroid production with drugs like trilostane (a 3β-hydroxysteroid dehydrogenase inhibitor) or indomethacin (a 3α-hydroxysteroid dehydrogenase inhibitor) completely nullified agmatine's effect. Seizures were associated with elevated progesterone levels and reduced allopregnanolone levels, which were normalized by agmatine treatment. Notably, agmatine exhibited seizure protection even in ovariectomized rats, affirming the involvement of neurosteroids in its anti-seizure effects in progesterone withdrawal increased seizure susceptibility. In conclusion, these findings emphasize central neurosteroid involvement in agmatine's pharmacological effects against seizure susceptibility in progesterone withdrawal in female rats.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100142"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000271/pdfft?md5=47fb1c1f560730271b01d86f590fb018&pid=1-s2.0-S2666459324000271-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141145063","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rapidly progressive seronegative immune-mediated neuropathies responded to “Remove and suppress” therapy with plasma exchange and B-cell suppression therapy","authors":"Stefanie Kar Yan Hung ,&nbsp;Daniel Tze Wei Yeap ,&nbsp;Thanusha Karunakaran ,&nbsp;Dhayalen Krishnan ,&nbsp;Sow Kuan Tee ,&nbsp;Fu Liong Hiew","doi":"10.1016/j.dscb.2024.100148","DOIUrl":"https://doi.org/10.1016/j.dscb.2024.100148","url":null,"abstract":"<div><p>Immune-mediated peripheral neuropathies are heterogeneous group of disorders due to the present of autoantibodies against peripheral nerve molecules located in node of Ranvier such as gangliosides and cell adhesion proteins or myelin components of peripheral nerves. Although the exact aetiology and pathophysiological mechanisms involved are not fully understood, both humoral and cellular immunity are likely playing a role in their pathogenesis. A proportion of patients present with clinical phenotype of rapidly progressive neuropathy refractory to conventional therapies but are lacking in identifiable or detectable antibodies. This makes diagnosis and treatment decision challenging.</p><p>We illustrate a patient with rapidly progressive seronegative immune-mediated neuropathy resembling autoimmune nodopathy (AN) associated with atypical features (prominent ataxia) and cranial involvement (facial and oropharyngeal weakness, dysgeusia), refractory to conventional therapies (IV immunoglobulin and corticosteroids) but responded to ‘remove and suppress’ treatment regime using therapeutic plasma exchange (TPE), followed by long-term B-cell suppressive therapy.</p></div>","PeriodicalId":72447,"journal":{"name":"Brain disorders (Amsterdam, Netherlands)","volume":"14 ","pages":"Article 100148"},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666459324000337/pdfft?md5=7db3053b424c0f1e530211338a548627&pid=1-s2.0-S2666459324000337-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141479479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}