{"title":"\"Crab sign\" in an ataxia patient with central nervous system Erdheim Chester disease: a novel neuroimaging finding with review of literature.","authors":"Farsana Mustafa, Chaithra Rajanna, Archita Makharia, Divyani Garg, Ayush Agarwal, Ajay Garg, Aanchal Kakkar, Shreya Ojha, Nishikant A Damle, Yamini Dharmashaktu, Divya M Radhakrishnan, Achal Kumar Srivastava","doi":"10.1007/s10072-025-08381-7","DOIUrl":"https://doi.org/10.1007/s10072-025-08381-7","url":null,"abstract":"<p><strong>Background: </strong>Erdheim-Chester disease (ECD) of the central nervous system is a neoplastic, non-Langerhans cell histiocytosis which can present with a multitude of symptoms. Intra-axial involvement commonly leads to a spastic ataxic syndrome, ophthalmoplegia, and diabetes insipidus while extra-axial involvement manifests as pachymeningitis.</p><p><strong>Objectives: </strong>To describe a novel imaging sign in Erdheim Chester Disease.</p><p><strong>Methods: </strong>We evaluated in detail a 55-year old lady with xanthelesmas and a progressive spastic ataxic syndrome over a 6 month period by history, examination, serological, radiological and histopathological investigations.</p><p><strong>Results: </strong>Our patient was diagnosed with Erdheim Chester disease based on histopathological evaluation. She had a novel neuroimaging finding of \"crab sign\" in the axial T2-FLAIR sections of her MRI brain.</p><p><strong>Conclusion: </strong>The \"crab sign\" has the potential to aid early diagnosis and enhance diagnostic yield of Erdheim Chester disease, facilitating earlier therapeutic intervention and better outcomes.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of IgG4-related spinal pachymeningitis with a large spinal cord cavity: case report and updated systematic review.","authors":"Jiaqian Zhu, Yanni Yu, Peng Sun, Liming Cao","doi":"10.1007/s10072-025-08369-3","DOIUrl":"https://doi.org/10.1007/s10072-025-08369-3","url":null,"abstract":"<p><strong>Background: </strong>IgG4-related spinal pachymeningitis (IgG4-RSP) is rare, particularly in cases where spinal cord cavities develop after the removal of thickened dura mater, making diagnosis challenging. A 31-year-old male presented with IgG4-RSP, and the clinical presentation, diagnostic imaging, pathology, treatment, and follow-up outcomes were documented. We also conducted a thorough review of the relevant literature.</p><p><strong>Case report: </strong>The patient presented with two-year progressive lower limb weakness. He experienced lower back pain, numbness in both legs, and urinary and defecatory difficulties without a notable medical history. Initial magnetic resonance imaging (MRI) revealed dura mater lesions and thoracic spinal cord compression, leading to surgery that did not improve the symptoms. Pathological examination confirmed IgG4-related hypertrophic pachymeningitis. Physical examination revealed a reduced pain sensation below both rib margins and a muscle strength score of 3/5 in the lower limbs. Thoracic MRI revealed significantly enlarged spinal cord cavities compared to previous scans. Methylprednisolone treatment partially improved symptoms. Subsequent efgartigimod alfa treatment did not alleviate the symptoms further. The patient exhibited significant neurological deficits.</p><p><strong>Literature review: </strong>Studies were identified through PubMed, resulting in 20 case reports that met the criteria and were pathologically confirmed. More than half of all patients with IgG4-RSP experience neck or back pain, with lesions typically found in the cervical spine and extending to the thoracic level.</p><p><strong>Conclusions: </strong>Caution should be exercised in the post-surgical spinal cord cavity. One-quarter of patients have a poor prognosis. Further research is needed to understand the pathophysiology of IgG4-RSP and develop specific treatments.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144718161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shree Rath, Dur E Sameen, Neha Waseem, Sahar Ahsan, Bushra Syed, Arham Khalid Farooq, Ahmad Kamal
{"title":"Addressing inequities in neurological mortality: trends and disparities in anoxic brain injury deaths, 1999-2023.","authors":"Shree Rath, Dur E Sameen, Neha Waseem, Sahar Ahsan, Bushra Syed, Arham Khalid Farooq, Ahmad Kamal","doi":"10.1007/s10072-025-08382-6","DOIUrl":"https://doi.org/10.1007/s10072-025-08382-6","url":null,"abstract":"<p><strong>Purpose: </strong>Despite the slowly declining prevalence of anoxic brain injury (ABI) due to newer drugs and guidelines, there still exists inequity in timely receipt of treatment. This study aims to provide a comprehensive evaluation of ABI mortality trends and sociodemographic disparities in the United States from 1999 to 2023.</p><p><strong>Methods: </strong>Mortality data were obtained from the CDC-WONDER Database. The study focused on ABI-related deaths among adults aged 35 to 85 + years between 1999 and 2023. Demographic and regional variables were analyzed, including gender, race/ethnicity, age groups, census region, state, urbanization, and place of death. Average annual percentage changes (AAPC) were calculated, and trends were evaluated using the Joinpoint Regression.</p><p><strong>Results: </strong>From 1999 to 2023, there were a total of 117,148 ABI-related deaths. The overall age-adjusted mortality rate (AAMR) showed a mild increase from 1999 to 2004, followed by a sharp increase until 2017, and a subsequent decline (APC: -3.10% [95% CI, -4.1% to -2.37%]. Males consistently exhibited higher mortality rates compared to females. Significant racial disparities were observed, with African Americans demonstrating higher AAMRs (7.67) than other racial groups. Both urban and rural areas experienced trends in ABI mortality, with rural areas showing higher AAMRs.</p><p><strong>Conclusion: </strong>Despite recent declines in overall ABI mortality trends, significant disparities persist across demographic and geographic lines. Targeted public health interventions are necessary to address risk factors leading to ABI, particularly among high-risk groups such as racial minorities and rural populations.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144718162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vincenzina Lo Re, Giuseppe Mamone, Alessandro Mattina
{"title":"Letter to Editor concerning \"rapidly progressive dementia in tacrolimus neurotoxicity: rare but reversible\".","authors":"Vincenzina Lo Re, Giuseppe Mamone, Alessandro Mattina","doi":"10.1007/s10072-025-08378-2","DOIUrl":"https://doi.org/10.1007/s10072-025-08378-2","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144708260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Central nervous system demyelination associated with biological agents used in the treatment of rheumatologic diseases: a retrospective cohort study.","authors":"Doruk Arslan, Basak Sayinalp-Arslan, Emre Bilgin, Pinar Acar-Ozen, Rahsan Gocmen, Ihsan Ertenli, Sedat Kiraz, Rana Karabudak, Umut Kalyoncu, Asli Tuncer","doi":"10.1007/s10072-025-08354-w","DOIUrl":"https://doi.org/10.1007/s10072-025-08354-w","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the annual incidence rate and clinical characteristics of central nervous system (CNS) demyelinating disease in patients who receive biologic agents for rheumatic diseases.</p><p><strong>Methods: </strong>Patients who received biologic agents due to rheumatic diseases were identified in a retrospective manner and data of the patients who developed CNS demyelinating disease were recorded.</p><p><strong>Results: </strong>4838 out of 5926 patients who attended regular follow-up visits, were included in the study. CNS demyelinating disease was observed in 7 patients for 19.391 patient-years. 4 of 7 were diagnosed with multiple sclerosis (MS). 6 out of 7 were under anti-TNF therapy and mean duration was 13 months under causative drugs. While annual incidence rate of CNS demyelinating disease was 36.1/100000 (95% CI: 17-75), annual multiple sclerosis incidence rate in rheumatologic patients exposed to biologic agents was 19.6/100000 (95% confidence interval 7-52).</p><p><strong>Conclusion: </strong>When compared with the data obtained in our cohort with annual incidence rates of multiple sclerosis in Europe, it is possible to conclude that there is an increase in the incidence of demyelinating events. Therefore, clinicians must be aware of neurological signs and symptoms in patients who receive biologic agents.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144699119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mahmoud A Farid, Mohamed G Zeinhom, Sherihan Rezk Ahmed, Ehab S Ramadan
{"title":"Factors influencing the occurrence and characteristics of white matter hyperintensities on the brain imaging of migraine patients.","authors":"Mahmoud A Farid, Mohamed G Zeinhom, Sherihan Rezk Ahmed, Ehab S Ramadan","doi":"10.1007/s10072-025-08373-7","DOIUrl":"https://doi.org/10.1007/s10072-025-08373-7","url":null,"abstract":"<p><strong>Background: </strong>White matter hyperintensities (WMHs) increase the burden of migraine on the patients' lives and leading to migraine-related poor outcomes, so we aim to evaluate the association of different neurophysiological, psychological, and clinical factors with the presence and characteristics of WMHs on the brain imaging of migraine patients.</p><p><strong>Methods: </strong>Our study included 300 migraine patients who underwent clinical, psychological, and neurophysiological assessment, including P300, which represented an event-related potential (ERP) component elicited in the decision-making process. The goal was to evaluate the association of these factors with the presence and characteristics of WMHs on the brain imaging of migraine patients.</p><p><strong>Results: </strong>older age (OR, 1.213; 95% CI, 1.149 to 1.787; P-value 0.02), P300 Fz latency (OR, 1.852; 95% CI, 1.374 to 2.314; P-value < 0.001), P300 Pz latency (OR, 1.823; 95% CI, 1.397 to 4.237; P-value < 0.001), migraine with aura (OR, 7.115; 95% CI, 3.874 to 14.254; P-value 0.01), chronic migraine (OR, 6.105; 95% CI, 3.364 to 8.057; P-value < 0.001), and Montreal cognitive assessment (MoCA) (OR, 0.543; 95% CI, 0.169 to 0.723; P-value < 0.001) were associated with developing WMHs on the brain MRI of migraine patients.</p><p><strong>Conclusion: </strong>Prolonged P300 latency, older age, lower MoCA score, chronic migraine, and migraine with aura were associated with the presence of WMHs on the brain imaging of migraine patients. In addition, longer P300 latency was positively correlated with WMH diameter, number, and Schelten's score. In contrast, higher P300 amplitude and higher MoCA score were negatively correlated with WMH diameter, number, and Schelten's score in migraine patients.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144691000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carolina Cutrona, Matteo Costanzo, Giorgio Leodori, Maria Ilenia De Bartolo, Agnese Liguori, Francesco Marchet, Marco Mancuso, Giorgio Vivacqua, Antonella Conte, Giovanni Fabbrini, Alfredo Berardelli, Daniele Belvisi
{"title":"The evolution of tremor in Parkinson's Disease: insights from a 4-year longitudinal assessment.","authors":"Carolina Cutrona, Matteo Costanzo, Giorgio Leodori, Maria Ilenia De Bartolo, Agnese Liguori, Francesco Marchet, Marco Mancuso, Giorgio Vivacqua, Antonella Conte, Giovanni Fabbrini, Alfredo Berardelli, Daniele Belvisi","doi":"10.1007/s10072-025-08363-9","DOIUrl":"https://doi.org/10.1007/s10072-025-08363-9","url":null,"abstract":"<p><strong>Introduction: </strong>While tremor is considered a cardinal motor sign in patients with Parkinson's disease (PD), little is known about the evolution of the different types of PD tremor over time.</p><p><strong>Aims: </strong>Our objectives were to assess the rate of progression of the various types of PD tremor over the disease course and to verify if the presence of different tremors is consistently associated with specific motor and non-motor burdens over time. Finally, we investigated whether the presence of different tremors can predict specific trajectories of disease progression.</p><p><strong>Methods: </strong>One hundred PD patients were enrolled and 73 completed a 4-year follow up. Clinical evaluations included the administration of standardized PD scales to assess the severity of motor and non-motor manifestations. The occurrence and severity of rest, re-emergent, and action tremors were accurately evaluated at baseline and 4 years later. Adjusted linear regression models were used to assess tremor type's influence on disease progression.</p><p><strong>Results: </strong>Tremor occurrence and severity decreased during PD progression, with a more significant reduction in action tremor compared to rest and re-emergent tremor. Patients with rest and re-emergent tremor had milder motor symptoms at baseline and milder motor and non-motor manifestations at follow-up. The presence of rest and re-emergent tremor predicted a lower progression of non-motor symptoms.</p><p><strong>Conclusions: </strong>This longitudinal study revealed that the various types of PD tremor have different evolutions over disease course, can predict distinct trajectories of disease progression and are underpinned by distinct pathophysiological mechanisms.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144691001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Enis Guso, Irene Volonghi, Sara Mariotto, Davide Arici, Emanuele Olivieri, Andrea Pilotto, Alessandro Padovani
{"title":"Possible autoimmune encephalitis in a patient with Good's syndrome.","authors":"Enis Guso, Irene Volonghi, Sara Mariotto, Davide Arici, Emanuele Olivieri, Andrea Pilotto, Alessandro Padovani","doi":"10.1007/s10072-025-08374-6","DOIUrl":"https://doi.org/10.1007/s10072-025-08374-6","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marco Bolchini, Giulia Ceccardi, Francesca Schiano di Cola, Michele Di Pasquale, Renata Rao, Alessandro Padovani
{"title":"Anti-CGRP monoclonal antibodies, migraine and mood disorders: an observational study.","authors":"Marco Bolchini, Giulia Ceccardi, Francesca Schiano di Cola, Michele Di Pasquale, Renata Rao, Alessandro Padovani","doi":"10.1007/s10072-025-08372-8","DOIUrl":"https://doi.org/10.1007/s10072-025-08372-8","url":null,"abstract":"<p><strong>Introduction: </strong>Migraine is frequently associated with psychiatric comorbidities, especially anxiety and depressive disorders, with related worse clinical outcome and chronicisation. Aim of the study was to assess the effectiveness of anti-CGRP antibodies in alleviating migraine-related symptoms and comorbid anxiety and depressive symptoms.</p><p><strong>Methods: </strong>Retrospective observational study including 206 migraine out-patients in treatment with anti-CGRP antibodies. Beck Depression Inventory (BDI) scale assessing depressive symptoms and Zung Anxiety Scale rating anxiety manifestations, were adopted. Patients were divided into two groups based on the severity of mood disturbances according to BDI score. Effectiveness outcomes (monthly headache days (MHDs), monthly migraine days (MMDs), analgesic consumption, Numerical Rating Scale (NRS), Migraine Disability Assessment (MIDAS) score, Headache Impact Test (HIT-6) score) and self-assessment anxiety and depression scales (BDI, Zung) were analysed after 3 and 6 months of treatment.</p><p><strong>Results: </strong>Effectiveness outcomes showed a significant clinical improvement at T3 (ΔMHD - 9.99 ± 7.82 in Group 1, -10.82 ± 7.62 in Group 2) and T6 (MHD - 10.30 ± 7.41 in Group 1, -10.61 ± 7.87 in Group 2), irrespective of the presence of associated mood symptoms. Treatment resulted in a notable improvement in anxiety and depressive manifestations, with significant reduction in BDI and Zung scales at T3 (ΔBDI - 1.30 ± 3.74 in Group 1, -6.35 ± 11.34 in Group 2) and T6 (ΔBDI - 1.67 ± 4.77 in Group 1, -8.06 ± 10.35 in Group 2).</p><p><strong>Conclusions: </strong>This study corroborates the effectiveness of anti-CGRP antibody treatment in migraine patients, irrespective of the presence of comorbid mood-related symptoms. Furthermore, the persistently favourable outcome in mood symptom severity and intensity throughout the course of treatment substantiates the beneficial impact of this therapeutic approach.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675358","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}