Objective: The primary objective of this study was to explore the clinical characteristics of apoplectic intratumoral hemorrhage in gliomas and offer insights for improving the diagnosis and treatment of this disease.
Methods: We analyzed the clinical data of 35 patients with glioma and hemorrhage. There were eight cases of multiple cerebral lobe involvement, and 22 cases involved a single lobe. Twenty-one patients had a preoperative Glasgow Coma Scale (GCS) score of ≥ 9 and had a craniotomy with tumor resection and hematoma evacuation after undergoing preoperative preparation. A total of 14 patients with GCS < 9, including one with thalamic hemorrhage breaking into the ventricles and acute obstructive hydrocephalus, underwent craniotomy for tumor resection after external ventricular drainage (EVD). One patient had combined thrombocytopenia, which was surgically treated after platelet levels were normalized through transfusion. The remaining 12 patients received immediate intervention in the form of craniotomy hematoma evacuation and tumor resection.
Results: We performed subtotal resection on three tumors of thalamic origin and two tumors of corpus callosum origin, but we were able to successfully resect all the tumors in other locations that were gross total resection Pathology results showed that 71.43% of cases accounted for WHO-grade 4 tumors. Among the 21 patients with a GCS score of ≥ 9, two died perioperatively. Fourteen patients had a GCS score < 9, of which eight patients died perioperatively.
Conclusions: Patients with a preoperative GCS score ≥ 9 who underwent subemergency surgery and received aggressive treatment showed a reasonable prognosis. We found their long-term outcomes to be correlated with the pathology findings. On the other hand, patients with a preoperative GCS score < 9 required emergency treatment and had a high perioperative mortality rate.
{"title":"Clinical features and treatment of apoplectic intratumoral hemorrhage of glioma.","authors":"Jia-Hua Zhou, Chao Wang, Di Yang, Ying-Xi Wu, Da-Yun Feng, Huaizhou Qin, Ju-Lei Wang, Ming-Hao Wei","doi":"10.1186/s12883-024-03753-6","DOIUrl":"10.1186/s12883-024-03753-6","url":null,"abstract":"<p><strong>Objective: </strong>The primary objective of this study was to explore the clinical characteristics of apoplectic intratumoral hemorrhage in gliomas and offer insights for improving the diagnosis and treatment of this disease.</p><p><strong>Methods: </strong>We analyzed the clinical data of 35 patients with glioma and hemorrhage. There were eight cases of multiple cerebral lobe involvement, and 22 cases involved a single lobe. Twenty-one patients had a preoperative Glasgow Coma Scale (GCS) score of ≥ 9 and had a craniotomy with tumor resection and hematoma evacuation after undergoing preoperative preparation. A total of 14 patients with GCS < 9, including one with thalamic hemorrhage breaking into the ventricles and acute obstructive hydrocephalus, underwent craniotomy for tumor resection after external ventricular drainage (EVD). One patient had combined thrombocytopenia, which was surgically treated after platelet levels were normalized through transfusion. The remaining 12 patients received immediate intervention in the form of craniotomy hematoma evacuation and tumor resection.</p><p><strong>Results: </strong>We performed subtotal resection on three tumors of thalamic origin and two tumors of corpus callosum origin, but we were able to successfully resect all the tumors in other locations that were gross total resection Pathology results showed that 71.43% of cases accounted for WHO-grade 4 tumors. Among the 21 patients with a GCS score of ≥ 9, two died perioperatively. Fourteen patients had a GCS score < 9, of which eight patients died perioperatively.</p><p><strong>Conclusions: </strong>Patients with a preoperative GCS score ≥ 9 who underwent subemergency surgery and received aggressive treatment showed a reasonable prognosis. We found their long-term outcomes to be correlated with the pathology findings. On the other hand, patients with a preoperative GCS score < 9 required emergency treatment and had a high perioperative mortality rate.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11267862/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141757187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Transitioning to end-of-life care and thereby changing the focus of treatment directives from life-sustaining treatment to comfort care is important for neurological patients in advanced stages. Late transition to end-of-life care for neurological patients has been described previously. To investigate whether previous treatment directives, primary medical diagnoses, and demographic factors predict the transition to end-of-life care and time to eventual death in patients with neurological diseases in an acute hospital setting. All consecutive health records of patients diagnosed with stroke, amyotrophic lateral sclerosis (ALS), and Parkinson’s disease or other extrapyramidal diseases (PDoed), who died in an acute neurological ward between January 2011 and August 2020 were retrieved retrospectively. Descriptive statistics and multivariate Cox regression were used to examine the timing of treatment directives and death in relation to medical diagnosis, age, gender, and marital status. A total of 271 records were involved in the analysis. Patients in all diagnostic categories had a treatment directive for end-of-life care, with patients with haemorrhagic stroke having the highest (92%) and patients with PDoed the lowest (73%) proportion. Cox regression identified that the likelihood of end-of-life care decision-making was related to advancing age (HR = 1.02, 95% CI: 1.007–1.039, P = 0.005), ischaemic stroke (HR = 1.64, 95% CI: 1.034–2.618, P = 0.036) and haemorrhagic stroke (HR = 2.04, 95% CI: 1.219–3.423, P = 0.007) diagnoses. End-of-life care decision occurred from four to twenty-two days after hospital admission. The time from end-of-life care decision to death was a median of two days. Treatment directives, demographic factors, and diagnostic categories did not increase the likelihood of death following an end-of-life care decision. Results show not only that neurological patients transit late to end-of-life care but that the timeframe of the decision differs between patients with acute neurological diseases and those with progressive neurological diseases, highlighting the particular significance of the short timeframe of patients with the progressive neurological diseases ALS and PDoed. Different trajectories of patients with neurological diseases at end-of-life should be further explored and clinical guidelines expanded to embrace the high diversity in neurological patients.
{"title":"Transition to end-of-life care in patients with neurological diseases in an acute hospital ward","authors":"Gudrun Jonsdottir, Erna Haraldsdottir, Runar Vilhjalmsson, Valgerdur Sigurdardottir, Haukur Hjaltason, Marianne Elisabeth Klinke, Gudny Bergthora Tryggvadottir, Helga Jonsdottir","doi":"10.1186/s12883-024-03768-z","DOIUrl":"https://doi.org/10.1186/s12883-024-03768-z","url":null,"abstract":"Transitioning to end-of-life care and thereby changing the focus of treatment directives from life-sustaining treatment to comfort care is important for neurological patients in advanced stages. Late transition to end-of-life care for neurological patients has been described previously. To investigate whether previous treatment directives, primary medical diagnoses, and demographic factors predict the transition to end-of-life care and time to eventual death in patients with neurological diseases in an acute hospital setting. All consecutive health records of patients diagnosed with stroke, amyotrophic lateral sclerosis (ALS), and Parkinson’s disease or other extrapyramidal diseases (PDoed), who died in an acute neurological ward between January 2011 and August 2020 were retrieved retrospectively. Descriptive statistics and multivariate Cox regression were used to examine the timing of treatment directives and death in relation to medical diagnosis, age, gender, and marital status. A total of 271 records were involved in the analysis. Patients in all diagnostic categories had a treatment directive for end-of-life care, with patients with haemorrhagic stroke having the highest (92%) and patients with PDoed the lowest (73%) proportion. Cox regression identified that the likelihood of end-of-life care decision-making was related to advancing age (HR = 1.02, 95% CI: 1.007–1.039, P = 0.005), ischaemic stroke (HR = 1.64, 95% CI: 1.034–2.618, P = 0.036) and haemorrhagic stroke (HR = 2.04, 95% CI: 1.219–3.423, P = 0.007) diagnoses. End-of-life care decision occurred from four to twenty-two days after hospital admission. The time from end-of-life care decision to death was a median of two days. Treatment directives, demographic factors, and diagnostic categories did not increase the likelihood of death following an end-of-life care decision. Results show not only that neurological patients transit late to end-of-life care but that the timeframe of the decision differs between patients with acute neurological diseases and those with progressive neurological diseases, highlighting the particular significance of the short timeframe of patients with the progressive neurological diseases ALS and PDoed. Different trajectories of patients with neurological diseases at end-of-life should be further explored and clinical guidelines expanded to embrace the high diversity in neurological patients.","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141739473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Caregivers’ knowledge and attitudes influence help-seeking behavior and treatment decisions of patients with epilepsy, which in turn significantly impacts epilepsy care. In Ethiopia, epilepsy is often misunderstood, associated with misconceptions and accompanied by persistent negative attitudes. The objective of this study is to assess the knowledge, attitude, and practice of caregivers of children with epilepsy. We conducted a hospital-based survey at the Yekatit 12 Hospital Pediatric Neurology Clinic, Addis Ababa, Ethiopia, between May and July 2022. We invited caregivers of children with epilepsy taking one or more daily anti-seizure medications to participate. Caregivers were invited to complete a structured questionnaire with guidance from a trained nurse to estimate knowledge and attitudes towards epilepsy and its treatment. Knowledge and attitudes were categorized as “good” and “favorable” (correct answers to ≥ 50% of questions) or “bad” and “unfavorable” (< 50% correct answers), respectively. Attitudes towards standard care versus non-standard (e.g., spiritual) care were also estimated. A total of 120 caregivers completed the questionnaire. Many caregivers were familiar with the term ‘epilepsy’, with more than half (51.7%) having heard or read about it previously. The reported causes of epilepsy varied, with birth injury being the most common cause (44 out of 120 caregivers). Notably, there was association between the caregiver’s gender and their knowledge score, with a p-value = 0.05. Caregivers exposed to information about epilepsy through hearing or reading demonstrated significantly higher levels of knowledge, with a p-value < 0.001. Additionally, knowing someone with epilepsy other than the index child was significantly associated with higher knowledge scores (p-value < 0.001). The study also revealed negative attitudes toward epilepsy: for example, 56.7% of surveyed caregivers believed it is unlikely that a child with epilepsy has normal cognitive abilities and 39.1% believed they should never be allowed to attend regular school. Additionally, a high proportion of caregivers (70%) sought alternative treatments (e.g., spiritual help) alongside standard medical care. A significant knowledge gap was identified among caregivers, revealing prevalent misconceptions and negative attitudes. Improving epilepsy awareness, attitudes, and practices among caregivers will potentially contribute to overall improved quality of life for children with epilepsy.
{"title":"Knowledge, attitudes, and practices of caregivers with children diagnosed with epilepsy attending a pediatric outpatient clinic: a descriptive, cross-sectional, questionnaire-based study in Addis Ababa, Ethiopia","authors":"Absalat Serawit Negussie, Mansour Fayz Dehan, Samuel Ayalew Mekonnen, Tesfaye Getaneh Zelleke","doi":"10.1186/s12883-024-03766-1","DOIUrl":"https://doi.org/10.1186/s12883-024-03766-1","url":null,"abstract":"Caregivers’ knowledge and attitudes influence help-seeking behavior and treatment decisions of patients with epilepsy, which in turn significantly impacts epilepsy care. In Ethiopia, epilepsy is often misunderstood, associated with misconceptions and accompanied by persistent negative attitudes. The objective of this study is to assess the knowledge, attitude, and practice of caregivers of children with epilepsy. We conducted a hospital-based survey at the Yekatit 12 Hospital Pediatric Neurology Clinic, Addis Ababa, Ethiopia, between May and July 2022. We invited caregivers of children with epilepsy taking one or more daily anti-seizure medications to participate. Caregivers were invited to complete a structured questionnaire with guidance from a trained nurse to estimate knowledge and attitudes towards epilepsy and its treatment. Knowledge and attitudes were categorized as “good” and “favorable” (correct answers to ≥ 50% of questions) or “bad” and “unfavorable” (< 50% correct answers), respectively. Attitudes towards standard care versus non-standard (e.g., spiritual) care were also estimated. A total of 120 caregivers completed the questionnaire. Many caregivers were familiar with the term ‘epilepsy’, with more than half (51.7%) having heard or read about it previously. The reported causes of epilepsy varied, with birth injury being the most common cause (44 out of 120 caregivers). Notably, there was association between the caregiver’s gender and their knowledge score, with a p-value = 0.05. Caregivers exposed to information about epilepsy through hearing or reading demonstrated significantly higher levels of knowledge, with a p-value < 0.001. Additionally, knowing someone with epilepsy other than the index child was significantly associated with higher knowledge scores (p-value < 0.001). The study also revealed negative attitudes toward epilepsy: for example, 56.7% of surveyed caregivers believed it is unlikely that a child with epilepsy has normal cognitive abilities and 39.1% believed they should never be allowed to attend regular school. Additionally, a high proportion of caregivers (70%) sought alternative treatments (e.g., spiritual help) alongside standard medical care. A significant knowledge gap was identified among caregivers, revealing prevalent misconceptions and negative attitudes. Improving epilepsy awareness, attitudes, and practices among caregivers will potentially contribute to overall improved quality of life for children with epilepsy.","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141739472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-22DOI: 10.1186/s12883-024-03748-3
Nils Jansen, Leonard Wulff, Moritz Conty, Wolf-Rüdiger Schäbitz, Randolf Klingebiel
CHANTER (Cerebellar Hippocampal and Basal Nuclei Transient Edema with Restricted diffusion) is a recently described syndrome occurring in the context of drug abuse. While clinical findings are rather unspecific (disorientation, unresponsiveness), MR imaging (MRI) discloses a characteristic pattern (restricted diffusion in the basal ganglia and hippocampi, cerebellar oedema and haemorrhage), allowing for timely diagnosis before complications such as cerebellar swelling and herniation do occur. Here we report a case of CHANTER primarily based on imaging findings, as there was no evidence of drug abuse on admission. A 62-year-old Patient was admitted to our hospital after being unresponsive at home. Prehospital intubation was performed, which limited neurological assessment. Under these circumstances no obvious symptoms could be determined, i.e. pupils were isocoric and responsive, and there were no signs of seizures. While the initial CT scan was unremarkable, the subsequent MRI scan showed a distinct imaging pattern: moderately enhancing areas in the basal ganglia and hippocampi with diffusion restriction, accompanied by cerebellar haemorrhage and oedema (Figs. 1 and 2). A comprehensive clinical and laboratory work-up was performed, including drug screening, spinal tap, Holter ECG, echocardiography and EEG. The only conspicuous anamnestic finding was a chronic pain syndrome whose medication had been supplemented with opioids two months previously. The opioid medication was discontinued, which led to a rapid improvement in the patient’s clinical condition without any further measures. The patient was able to leave the intensive care unit and was discharged 10 days after admission without persistent neurological deficits. Familiarity with typical MRI patterns of toxic encephalopathy in patients from high-risk groups, such as drug abusers, is crucial in emergency neuroradiology. In the presence of typical MRI findings, CHANTER syndrome should be included in the differential diagnosis, even if there is no history of drug abuse, to avoid delay in diagnosis and treatment.
{"title":"CHANTER syndrome in the context of pain medication: a case report","authors":"Nils Jansen, Leonard Wulff, Moritz Conty, Wolf-Rüdiger Schäbitz, Randolf Klingebiel","doi":"10.1186/s12883-024-03748-3","DOIUrl":"https://doi.org/10.1186/s12883-024-03748-3","url":null,"abstract":"CHANTER (Cerebellar Hippocampal and Basal Nuclei Transient Edema with Restricted diffusion) is a recently described syndrome occurring in the context of drug abuse. While clinical findings are rather unspecific (disorientation, unresponsiveness), MR imaging (MRI) discloses a characteristic pattern (restricted diffusion in the basal ganglia and hippocampi, cerebellar oedema and haemorrhage), allowing for timely diagnosis before complications such as cerebellar swelling and herniation do occur. Here we report a case of CHANTER primarily based on imaging findings, as there was no evidence of drug abuse on admission. A 62-year-old Patient was admitted to our hospital after being unresponsive at home. Prehospital intubation was performed, which limited neurological assessment. Under these circumstances no obvious symptoms could be determined, i.e. pupils were isocoric and responsive, and there were no signs of seizures. While the initial CT scan was unremarkable, the subsequent MRI scan showed a distinct imaging pattern: moderately enhancing areas in the basal ganglia and hippocampi with diffusion restriction, accompanied by cerebellar haemorrhage and oedema (Figs. 1 and 2). A comprehensive clinical and laboratory work-up was performed, including drug screening, spinal tap, Holter ECG, echocardiography and EEG. The only conspicuous anamnestic finding was a chronic pain syndrome whose medication had been supplemented with opioids two months previously. The opioid medication was discontinued, which led to a rapid improvement in the patient’s clinical condition without any further measures. The patient was able to leave the intensive care unit and was discharged 10 days after admission without persistent neurological deficits. Familiarity with typical MRI patterns of toxic encephalopathy in patients from high-risk groups, such as drug abusers, is crucial in emergency neuroradiology. In the presence of typical MRI findings, CHANTER syndrome should be included in the differential diagnosis, even if there is no history of drug abuse, to avoid delay in diagnosis and treatment.","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141739548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-22DOI: 10.1186/s12883-024-03767-0
Geraldine O’Callaghan, Martin Fahy, Sigrid O’Meara, Sebastian Lindblom, Lena von Koch, Peter Langhorne, Rose Galvin, Frances Horgan
Transitioning home from the structured hospital setting poses challenges for people with stroke (PWS) and their caregivers (CGs), as they navigate through complex uncertainties. There are gaps in our understanding of appropriate support interventions for managing the transition home. In this qualitative study, we explored the perspectives of PWS and their CGs regarding their support experiences and preferences during this period. Between November 2022 and March 2023, and within six months of hospital discharge, audio-recorded, semi-structured interviews were conducted with PWS and CGs. All interviews were transcribed, imported into NVivo software, and analysed using reflexive thematic analysis. Sixteen interviews were conducted, nine with PWS and seven with CGs. Four themes relevant to their collective experiences and preferences were identified: (i) Need for tailored information-sharing, at the right time, and in the right setting; (ii) The importance of emotional support; (iii) Left in limbo, (iv) Inequity of access. Experiences depict issues such as insufficient information-sharing, communication gaps, and fragmented and inequitable care; while a multi-faceted approach is desired to ease anxiety and uncertainty, minimise delays, and optimise recovery and participation during transition. Our findings highlight that regardless of the discharge route, and even with formal support systems in place, PWS and families encounter challenges during the transition period. The experiences of support at this transition and the preferences of PWS and CGs during this important period highlights the need for better care co-ordination, early and ongoing emotional support, and equitable access to tailored services and support. Experiences are likely to be improved by implementing a partnership approach with improved collaboration, including joint goal-setting, between PWS, CGs, healthcare professionals and support organisations.
{"title":"Experiences and preferences of people with stroke and caregivers, around supports provided at the transition from hospital to home: a qualitative descriptive study","authors":"Geraldine O’Callaghan, Martin Fahy, Sigrid O’Meara, Sebastian Lindblom, Lena von Koch, Peter Langhorne, Rose Galvin, Frances Horgan","doi":"10.1186/s12883-024-03767-0","DOIUrl":"https://doi.org/10.1186/s12883-024-03767-0","url":null,"abstract":"Transitioning home from the structured hospital setting poses challenges for people with stroke (PWS) and their caregivers (CGs), as they navigate through complex uncertainties. There are gaps in our understanding of appropriate support interventions for managing the transition home. In this qualitative study, we explored the perspectives of PWS and their CGs regarding their support experiences and preferences during this period. Between November 2022 and March 2023, and within six months of hospital discharge, audio-recorded, semi-structured interviews were conducted with PWS and CGs. All interviews were transcribed, imported into NVivo software, and analysed using reflexive thematic analysis. Sixteen interviews were conducted, nine with PWS and seven with CGs. Four themes relevant to their collective experiences and preferences were identified: (i) Need for tailored information-sharing, at the right time, and in the right setting; (ii) The importance of emotional support; (iii) Left in limbo, (iv) Inequity of access. Experiences depict issues such as insufficient information-sharing, communication gaps, and fragmented and inequitable care; while a multi-faceted approach is desired to ease anxiety and uncertainty, minimise delays, and optimise recovery and participation during transition. Our findings highlight that regardless of the discharge route, and even with formal support systems in place, PWS and families encounter challenges during the transition period. The experiences of support at this transition and the preferences of PWS and CGs during this important period highlights the need for better care co-ordination, early and ongoing emotional support, and equitable access to tailored services and support. Experiences are likely to be improved by implementing a partnership approach with improved collaboration, including joint goal-setting, between PWS, CGs, healthcare professionals and support organisations.","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141739474","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-22DOI: 10.1186/s12883-024-03761-6
Valentin Loser, Amandine Segot, Laurence de Leval, Bettina Bisig, Jean-Philippe Brouland, Ekkehard Hewer, Carmen Barcena, Andreas F. Hottinger, Caroline Pot
Diagnosis of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is challenging and often delayed. MRI imaging, CSF cytology and flow cytometry have a low sensitivity and even brain biopsies can be misleading. We report three cases of PCNSL with various clinical presentation and radiological findings where the diagnosis was suggested by novel CSF biomarkers and subsequently confirmed by brain biopsy or autopsy. Case presentations. The first case is a 79-year-old man with severe neurocognitive dysfunction and static ataxia evolving over 5 months. Brain MRI revealed a nodular ventriculitis. An open brain biopsy was inconclusive. The second case is a 60-year-old woman with progressive sensory symptoms in all four limbs, evolving over 1 year. Brain and spinal MRI revealed asymmetric T2 hyperintensities of the corpus callosum, corona radiata and corticospinal tracts. The third case is a 72-year-old man recently diagnosed with primary vitreoretinal lymphoma of the right eye. A follow-up brain MRI performed 4 months after symptom onset revealed a T2 hyperintense fronto-sagittal lesion, with gadolinium uptake and perilesional edema. In all three cases, CSF flow cytometry and cytology were negative. Mutation analysis on the CSF (either by digital PCR or by next generation sequencing) identified the MYD88 L265P hotspot mutation in all three cases. A B-cell clonality study, performed in case 1 and 2, identified a monoclonal rearrangement of the immunoglobulin light chain lambda (IGL) and kappa (IGK) gene. CSF CXCL-13 and IL-10 levels were high in all three cases, and IL-10/IL-6 ratio was high in two. Diagnosis of PCNSL was later confirmed by autopsy in case 1, and by brain biopsy in case 2 and 3. Taken together, 5 CSF biomarkers (IL-10, IL-10/IL-6 ratio, CXCL13, MYD88 mutation and monoclonal IG gene rearrangements) were strongly indicative of a PCNSL. Using innovative CSF biomarkers can be sensitive and complementary to traditional CSF analysis and brain biopsy in the diagnosis of PCNSL, potentially allowing for earlier diagnosis and treatment.
{"title":"Primary diffuse large B-cell lymphoma of the central nervous system identified with CSF biomarkers","authors":"Valentin Loser, Amandine Segot, Laurence de Leval, Bettina Bisig, Jean-Philippe Brouland, Ekkehard Hewer, Carmen Barcena, Andreas F. Hottinger, Caroline Pot","doi":"10.1186/s12883-024-03761-6","DOIUrl":"https://doi.org/10.1186/s12883-024-03761-6","url":null,"abstract":"Diagnosis of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is challenging and often delayed. MRI imaging, CSF cytology and flow cytometry have a low sensitivity and even brain biopsies can be misleading. We report three cases of PCNSL with various clinical presentation and radiological findings where the diagnosis was suggested by novel CSF biomarkers and subsequently confirmed by brain biopsy or autopsy. Case presentations. The first case is a 79-year-old man with severe neurocognitive dysfunction and static ataxia evolving over 5 months. Brain MRI revealed a nodular ventriculitis. An open brain biopsy was inconclusive. The second case is a 60-year-old woman with progressive sensory symptoms in all four limbs, evolving over 1 year. Brain and spinal MRI revealed asymmetric T2 hyperintensities of the corpus callosum, corona radiata and corticospinal tracts. The third case is a 72-year-old man recently diagnosed with primary vitreoretinal lymphoma of the right eye. A follow-up brain MRI performed 4 months after symptom onset revealed a T2 hyperintense fronto-sagittal lesion, with gadolinium uptake and perilesional edema. In all three cases, CSF flow cytometry and cytology were negative. Mutation analysis on the CSF (either by digital PCR or by next generation sequencing) identified the MYD88 L265P hotspot mutation in all three cases. A B-cell clonality study, performed in case 1 and 2, identified a monoclonal rearrangement of the immunoglobulin light chain lambda (IGL) and kappa (IGK) gene. CSF CXCL-13 and IL-10 levels were high in all three cases, and IL-10/IL-6 ratio was high in two. Diagnosis of PCNSL was later confirmed by autopsy in case 1, and by brain biopsy in case 2 and 3. Taken together, 5 CSF biomarkers (IL-10, IL-10/IL-6 ratio, CXCL13, MYD88 mutation and monoclonal IG gene rearrangements) were strongly indicative of a PCNSL. Using innovative CSF biomarkers can be sensitive and complementary to traditional CSF analysis and brain biopsy in the diagnosis of PCNSL, potentially allowing for earlier diagnosis and treatment.","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141739549","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Treponema pallidum can invade the central nervous system (CNS) early in its infection, causing neurosyphilis. Neurosyphilis typically presents with meningovasculitis in the acute or subacute phase, while tabes dorsalis and dementia paralytica are classical conditions in the later stages. However, syphilis is often misdiagnosed as other conditions such as tumors or autoimmune diseases including vasculitis and encephalitis, which is why the condition is known as "The Great Mimicker." The increasing incidence of syphilis in recent years emphasizes the importance of early diagnosis and treatment; however, its multiple clinical manifestations impose diagnostic challenges for clinicians because it resembles other diseases. In this case series, we present the impressive manifestations of neurosyphilis through three unique radiological presentations.
Case presentation: Case 1 details optic nerve involvement in an HIV-positive male, where MRI and fundoscopic findings confirmed syphilitic optic neuritis. Case 2 describes a patient in her pregnancy initially suspected of acoustic neuroma on MRI, later diagnosed with syphilitic gumma affecting the inner ear canal. Case 3 is a young male with clinical features mimicking temporal arteritis, ultimately identified as skull osteomyelitis secondarily causing inflammation of the musculus temporalis and meningitis.
Conclusions: These cases underscore the necessity of considering syphilis in differential diagnoses, given the diversity of its clinical presentations. Radiology plays an important role in avoiding unnecessary interventions. The increasing prevalence of recurrent syphilis imposes diagnostic challenges, emphasizing the importance of the early diagnosis and treatment of neurosyphilis by clinicians.
背景:苍白螺旋体可在感染早期侵入中枢神经系统(CNS),导致神经梅毒。神经梅毒在急性或亚急性期通常表现为脑膜血管炎,而在晚期则表现为背痛和麻痹性痴呆。然而,梅毒常常被误诊为其他疾病,如肿瘤或自身免疫性疾病,包括血管炎和脑炎,因此这种疾病被称为 "伟大的模仿者"。近年来,梅毒的发病率不断上升,强调了早期诊断和治疗的重要性;然而,由于梅毒的多种临床表现与其他疾病相似,给临床医生的诊断带来了挑战。在这组病例中,我们通过三种独特的放射学表现,介绍了神经梅毒令人印象深刻的表现:病例 1 详细描述了一名 HIV 阳性男性的视神经受累情况,核磁共振成像和眼底镜检查结果证实了梅毒性视神经炎。病例 2 描述了一名妊娠期患者,核磁共振成像最初怀疑其患有听神经瘤,后来确诊为梅毒性牙龈瘤累及内耳道。病例 3 是一名年轻男性,其临床特征类似颞动脉炎,最终被确定为颅骨骨髓炎,继而引起颞肌炎和脑膜炎:鉴于梅毒临床表现的多样性,这些病例强调了在鉴别诊断中考虑梅毒的必要性。放射学在避免不必要的干预方面发挥着重要作用。复发性梅毒的发病率越来越高,给诊断带来了挑战,强调了临床医生早期诊断和治疗神经梅毒的重要性。
{"title":"Novel and characteristic radiological features of neurosyphilis: a case series.","authors":"Kenji Ohira, Nanako Hashimoto, Daisuke Kanai, Yukio Inoue","doi":"10.1186/s12883-024-03762-5","DOIUrl":"10.1186/s12883-024-03762-5","url":null,"abstract":"<p><strong>Background: </strong>Treponema pallidum can invade the central nervous system (CNS) early in its infection, causing neurosyphilis. Neurosyphilis typically presents with meningovasculitis in the acute or subacute phase, while tabes dorsalis and dementia paralytica are classical conditions in the later stages. However, syphilis is often misdiagnosed as other conditions such as tumors or autoimmune diseases including vasculitis and encephalitis, which is why the condition is known as \"The Great Mimicker.\" The increasing incidence of syphilis in recent years emphasizes the importance of early diagnosis and treatment; however, its multiple clinical manifestations impose diagnostic challenges for clinicians because it resembles other diseases. In this case series, we present the impressive manifestations of neurosyphilis through three unique radiological presentations.</p><p><strong>Case presentation: </strong>Case 1 details optic nerve involvement in an HIV-positive male, where MRI and fundoscopic findings confirmed syphilitic optic neuritis. Case 2 describes a patient in her pregnancy initially suspected of acoustic neuroma on MRI, later diagnosed with syphilitic gumma affecting the inner ear canal. Case 3 is a young male with clinical features mimicking temporal arteritis, ultimately identified as skull osteomyelitis secondarily causing inflammation of the musculus temporalis and meningitis.</p><p><strong>Conclusions: </strong>These cases underscore the necessity of considering syphilis in differential diagnoses, given the diversity of its clinical presentations. Radiology plays an important role in avoiding unnecessary interventions. The increasing prevalence of recurrent syphilis imposes diagnostic challenges, emphasizing the importance of the early diagnosis and treatment of neurosyphilis by clinicians.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11264952/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-17DOI: 10.1186/s12883-024-03731-y
Markus Miedl, Philipp Baumgartner, Leah Raffaela Disse, Konrad Peter Weber, Heiko Pohl, Susanne Wegener
Background: The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported.
Case presentation: This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome.
Conclusions: This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.
{"title":"Harlequin syndrome in a patient with probable hemicrania continua and exertional headache - is there a link? a case report.","authors":"Markus Miedl, Philipp Baumgartner, Leah Raffaela Disse, Konrad Peter Weber, Heiko Pohl, Susanne Wegener","doi":"10.1186/s12883-024-03731-y","DOIUrl":"10.1186/s12883-024-03731-y","url":null,"abstract":"<p><strong>Background: </strong>The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported.</p><p><strong>Case presentation: </strong>This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome.</p><p><strong>Conclusions: </strong>This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11253322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141632733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-16DOI: 10.1186/s12883-024-03741-w
Qian Wu, Wenjie He, Chenyuan Liu, Xiaolin Yang, Jiakuan Chen, Boyan Xu, Xi Zhou, Guodong Huang, Jun Xia
Background: To investigate the association between white matter changes and ventricular expansion in idiopathic normal pressure hydrocephalus (iNPH) based on diffusion spectrum imaging (DSI).
Methods: We included 32 patients with iNPH who underwent DSI using a 3T MRI scanner. The lateral ventricles were manually segmented, and ventricular volumes were measured. Two methods were utilised in the study: manual region-of-interest (ROI) delineation and tract diffusion profile analysis. General fractional anisotropy (GFA) and fractional anisotropy (FA) were extracted in different white matter regions, including the bilateral internal capsule (anterior and posterior limbs) and corpus callosum (body, genu, and splenium) with manual ROI delineation. The 18 main tracts in the brain of each patient were extracted; the diffusion metrics of 100 equidistant nodes on each fibre were calculated, and Spearman's correlation coefficient was used to determine the correlation between diffusion measures and ventricular volume of iNPH patients.
Results: The GFA and FA of all ROI showed no significant correlation with lateral ventricular volume. However, in the tract diffusion profile analysis, lateral ventricular volume was positively correlated with part of the cingulum bundle, left corticospinal tract, and bilateral thalamic radiation posterior, whereas it was negatively correlated with the bilateral cingulum parahippocampal (all p < 0.05).
Conclusions: The effect of ventricular enlargement in iNPH on some white matter fibre tracts around the ventricles was limited and polarizing, and most white matter fibre tract integrity changes were not associated with ventricular enlargement; this reflects that multiple pathological mechanisms may have been combined to cause white matter alterations in iNPH.
背景:基于弥散谱成像(DSI)研究特发性正常压力脑积水(iNPH)患者白质变化与脑室扩张之间的关联:根据弥散谱成像(DSI)研究特发性正常压力脑积水(iNPH)患者白质变化与脑室扩张之间的关系:我们纳入了32名使用3T磁共振成像扫描仪进行DSI检查的特发性正常压力脑积水患者。人工分割侧脑室并测量脑室容积。研究中使用了两种方法:人工兴趣区(ROI)划分和束扩散轮廓分析。通过手动 ROI 划分,提取了不同白质区域的一般分数各向异性(GFA)和分数各向异性(FA),包括双侧内囊(前肢和后肢)和胼胝体(体、属和脾)。提取每位患者大脑中的18条主要纤维束,计算每条纤维上100个等距节点的扩散指标,并利用斯皮尔曼相关系数确定扩散指标与iNPH患者脑室容积的相关性:结果:所有 ROI 的 GFA 和 FA 与侧脑室容积无明显相关性。然而,在束扩散轮廓分析中,侧脑室容积与部分脑室束、左侧皮质脊髓束和双侧丘脑辐射后部呈正相关,而与双侧脑室旁呈负相关(均为 p):iNPH患者脑室扩大对脑室周围部分白质纤维束的影响是有限的,而且是两极化的,大多数白质纤维束的完整性变化与脑室扩大无关;这反映出多种病理机制可能共同导致了iNPH患者白质的改变。
{"title":"Diffusion spectrum imaging in patients with idiopathic normal pressure hydrocephalus: correlation with ventricular enlargement.","authors":"Qian Wu, Wenjie He, Chenyuan Liu, Xiaolin Yang, Jiakuan Chen, Boyan Xu, Xi Zhou, Guodong Huang, Jun Xia","doi":"10.1186/s12883-024-03741-w","DOIUrl":"10.1186/s12883-024-03741-w","url":null,"abstract":"<p><strong>Background: </strong>To investigate the association between white matter changes and ventricular expansion in idiopathic normal pressure hydrocephalus (iNPH) based on diffusion spectrum imaging (DSI).</p><p><strong>Methods: </strong>We included 32 patients with iNPH who underwent DSI using a 3T MRI scanner. The lateral ventricles were manually segmented, and ventricular volumes were measured. Two methods were utilised in the study: manual region-of-interest (ROI) delineation and tract diffusion profile analysis. General fractional anisotropy (GFA) and fractional anisotropy (FA) were extracted in different white matter regions, including the bilateral internal capsule (anterior and posterior limbs) and corpus callosum (body, genu, and splenium) with manual ROI delineation. The 18 main tracts in the brain of each patient were extracted; the diffusion metrics of 100 equidistant nodes on each fibre were calculated, and Spearman's correlation coefficient was used to determine the correlation between diffusion measures and ventricular volume of iNPH patients.</p><p><strong>Results: </strong>The GFA and FA of all ROI showed no significant correlation with lateral ventricular volume. However, in the tract diffusion profile analysis, lateral ventricular volume was positively correlated with part of the cingulum bundle, left corticospinal tract, and bilateral thalamic radiation posterior, whereas it was negatively correlated with the bilateral cingulum parahippocampal (all p < 0.05).</p><p><strong>Conclusions: </strong>The effect of ventricular enlargement in iNPH on some white matter fibre tracts around the ventricles was limited and polarizing, and most white matter fibre tract integrity changes were not associated with ventricular enlargement; this reflects that multiple pathological mechanisms may have been combined to cause white matter alterations in iNPH.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11251323/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141626000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-15DOI: 10.1186/s12883-024-03751-8
Juan Yang, Lu Liu, Jiahui Peng, Boya Ma, Xiao Yang
Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.
类风湿性脑膜炎(RM)是类风湿性关节炎(RA)的一种罕见关节外表现,已被越来越多的神经科医生所认识。然而,其临床表现的多样性给诊断带来了困难。RM尚无统一的诊断标准,其与RA的关系也有待进一步研究。在此,我们报告了两例无 RA 病史的 RM 患者。第一例患者是一名80岁的女性,突发单侧肢体无力,脑MR显示右侧额顶叶脑膜异常信号。影像学检查排除了蛛网膜下腔出血,脑脊液(CSF)检查排除了感染性脑膜炎。该患者被诊断为 RM,她的 CCP 和 AKA(RA 的标志物)水平升高,但没有该病的病史或其他临床表现。另一个病例是一名 65 岁的男性,因贝尔麻痹住院。我们在对他进行常规检查时发现,他的颅内影像学改变与RM的特征高度一致。除了左侧外周面部麻痹外,患者没有其他神经系统体征或症状,也没有 RA 病史。经过仔细的体格检查,我们没有发现与 RA 相符的关节或其他表现或血清学异常(RF、CCP、AKA 等)。然而,在排除感染性脑膜炎并考虑到患者独特的影像学结果后,我们诊断他患有RM。我们报告了这两个病例,作为RM临床诊断和治疗的参考,并对我们的理由进行了讨论。
{"title":"Rheumatoid meningitis in the absence of rheumatoid arthritis: 2 cases.","authors":"Juan Yang, Lu Liu, Jiahui Peng, Boya Ma, Xiao Yang","doi":"10.1186/s12883-024-03751-8","DOIUrl":"10.1186/s12883-024-03751-8","url":null,"abstract":"<p><p>Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.</p>","PeriodicalId":9170,"journal":{"name":"BMC Neurology","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11247829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141619378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}