Pub Date : 2024-05-03DOI: 10.1007/s11940-024-00795-6
Fazladin T. Temurov, Asel A. Biseytova, Bakhytkul A. Ernazarova, Bibifatima D. Mukhambetova, Asan S. Ubaydullaev
Purpose of Review
The research aims to determine the effectiveness of the main approaches in the diagnosis and treatment of trigeminal neuralgia. Analysis, synthesis, comparison, and generalisation were used as methods of studying the object of research.
Recent Findings
According to the results of the study, the most effective method of diagnosis was found to be examination and history taking. Correct performance of these actions allows to find out whether the nature of the patient’s pain is a symptom of trigeminal neuralgia. For this purpose, the location, nature, and triggering factors of the pain are determined. At the same time, pain characteristics are compared to exclude other diseases that are also accompanied by pain in the face. Among the instrumental diagnostic methods, magnetic resonance imaging has proven to be the most effective, allowing to determine the form of neuralgia and its causes. To determine the effectiveness of treatment methods, the parameters of pain relief rate, duration of the therapeutic effect and the presence of side effects were used. The most effective approach in the conservative treatment of trigeminal neuralgia was determined to be a complex drug therapy, including anticonvulsants, muscle relaxants, antispasmodics, and physiotherapy procedures. The most effective method of surgical intervention is microvascular decompression, which provides immediate pain relief in most cases and has the longest-lasting effect compared to other methods.
Summary
Rhizotomy was defined as a less effective procedure, but its advantage was its minimally invasive nature. Even though trigeminal neuralgia is a recurrent disease, the right treatment approach can maximise the duration of remission and return the patient to a normal lifestyle.
{"title":"The Efficiency of Established Diagnostic and Therapeutic Approaches in the Treatment of Trigeminal Neuralgia","authors":"Fazladin T. Temurov, Asel A. Biseytova, Bakhytkul A. Ernazarova, Bibifatima D. Mukhambetova, Asan S. Ubaydullaev","doi":"10.1007/s11940-024-00795-6","DOIUrl":"https://doi.org/10.1007/s11940-024-00795-6","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of Review</h3><p>The research aims to determine the effectiveness of the main approaches in the diagnosis and treatment of trigeminal neuralgia. Analysis, synthesis, comparison, and generalisation were used as methods of studying the object of research.</p><h3 data-test=\"abstract-sub-heading\">Recent Findings</h3><p>According to the results of the study, the most effective method of diagnosis was found to be examination and history taking. Correct performance of these actions allows to find out whether the nature of the patient’s pain is a symptom of trigeminal neuralgia. For this purpose, the location, nature, and triggering factors of the pain are determined. At the same time, pain characteristics are compared to exclude other diseases that are also accompanied by pain in the face. Among the instrumental diagnostic methods, magnetic resonance imaging has proven to be the most effective, allowing to determine the form of neuralgia and its causes. To determine the effectiveness of treatment methods, the parameters of pain relief rate, duration of the therapeutic effect and the presence of side effects were used. The most effective approach in the conservative treatment of trigeminal neuralgia was determined to be a complex drug therapy, including anticonvulsants, muscle relaxants, antispasmodics, and physiotherapy procedures. The most effective method of surgical intervention is microvascular decompression, which provides immediate pain relief in most cases and has the longest-lasting effect compared to other methods.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Rhizotomy was defined as a less effective procedure, but its advantage was its minimally invasive nature. Even though trigeminal neuralgia is a recurrent disease, the right treatment approach can maximise the duration of remission and return the patient to a normal lifestyle.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"14 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140883600","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}
Pub Date : 2024-04-19DOI: 10.1007/s11940-024-00793-8
Maximiliano A. Hawkes, Alejandro A. Rabinstein
Purpose of review
To provide an updated summary on the diagnosis and treatment of patients with malignant cerebral edema after ischemic stroke.
Recent findings
The risk of malignant middle cerebral artery (MCA) stroke is highest in young patients with large vessel occlusion and unsuccessful revascularization. Several scores are available for risk stratification. Treatment includes supportive care, close neurologic monitoring, and hyperosmolar therapy. Yet, the main therapeutic decision is whether to proceed with decompressive craniectomy. Multiple randomized clinical trials and several meta-analyses have demonstrated that decompressive hemicraniectomy is the single most important intervention associated with survival. Survivors may face severe disability regardless of surgical treatment, and the definition of acceptable outcome in this context remains elusive.
Summary
Malignant MCA infarcts are life-threatening and invariably cause disability, most often severe. Neurologic deterioration requires airway management and hyperosmolar therapy. Decompressive hemicraniectomy is a lifesaving procedure; approximately 50% of surgically treated patients younger than 60 years can regain independent ambulation, and one nearly in five may become functionally independent at 1 year. Older patients face a much worse functional prognosis; surgical decisions in these patients should be assessed case by case.
{"title":"Treatment of Malignant Cerebral Edema in Acute Ischemic Stroke","authors":"Maximiliano A. Hawkes, Alejandro A. Rabinstein","doi":"10.1007/s11940-024-00793-8","DOIUrl":"https://doi.org/10.1007/s11940-024-00793-8","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of review</h3><p>To provide an updated summary on the diagnosis and treatment of patients with malignant cerebral edema after ischemic stroke.</p><h3 data-test=\"abstract-sub-heading\">Recent findings</h3><p>The risk of malignant middle cerebral artery (MCA) stroke is highest in young patients with large vessel occlusion and unsuccessful revascularization. Several scores are available for risk stratification. Treatment includes supportive care, close neurologic monitoring, and hyperosmolar therapy. Yet, the main therapeutic decision is whether to proceed with decompressive craniectomy. Multiple randomized clinical trials and several meta-analyses have demonstrated that decompressive hemicraniectomy is the single most important intervention associated with survival. Survivors may face severe disability regardless of surgical treatment, and the definition of acceptable outcome in this context remains elusive.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Malignant MCA infarcts are life-threatening and invariably cause disability, most often severe. Neurologic deterioration requires airway management and hyperosmolar therapy. Decompressive hemicraniectomy is a lifesaving procedure; approximately 50% of surgically treated patients younger than 60 years can regain independent ambulation, and one nearly in five may become functionally independent at 1 year. Older patients face a much worse functional prognosis; surgical decisions in these patients should be assessed case by case.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"3 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140623403","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}
Pub Date : 2024-04-02DOI: 10.1007/s11940-024-00790-x
Amanda X. Y. Chin, Zhi Xuan Quak, Yee Cheun Chan, Amy M. L. Quek, Kay W. P. Ng
Purpose of review
This review aims to provide a summary of the pathophysiology, clinical presentation and management options for facioscapulohumeral dystrophy (FSHD). We discuss current management options and delve into updates about developments in targeted therapy.
Recent findings
New breakthroughs in FSHD research have led to a further understanding of aberrant DUX4 protein expression in the underlying pathophysiology of FSHD. This has paved the way for the development of targeted therapies aimed at targeting DUX4 expression or its downstream effects. Therapeutic strategies for FSHD primarily target DUX4 through three main avenues: small molecules, antisense oligonucleotide therapeutics and CRISPR-based approaches. This review discusses these strategies further. Presently, all prospective targeted therapies are in the pre-clinical phase, except for losmapimod, which is currently undergoing a phase 3 clinical trial.
Summary
Given the absence of approved disease-modifying treatments for FSHD, the primary approach for management currently involves multidisciplinary supportive measures which are limited. Recent developments in the form of targeted therapies and strategies for the definitive treatment of FSHD indicate a promising era.
{"title":"Updates on Facioscapulohumeral Muscular Dystrophy (FSHD)","authors":"Amanda X. Y. Chin, Zhi Xuan Quak, Yee Cheun Chan, Amy M. L. Quek, Kay W. P. Ng","doi":"10.1007/s11940-024-00790-x","DOIUrl":"https://doi.org/10.1007/s11940-024-00790-x","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of review</h3><p>This review aims to provide a summary of the pathophysiology, clinical presentation and management options for facioscapulohumeral dystrophy (FSHD). We discuss current management options and delve into updates about developments in targeted therapy.</p><h3 data-test=\"abstract-sub-heading\">Recent findings</h3><p>New breakthroughs in FSHD research have led to a further understanding of aberrant DUX4 protein expression in the underlying pathophysiology of FSHD. This has paved the way for the development of targeted therapies aimed at targeting DUX4 expression or its downstream effects. Therapeutic strategies for FSHD primarily target DUX4 through three main avenues: small molecules, antisense oligonucleotide therapeutics and CRISPR-based approaches. This review discusses these strategies further. Presently, all prospective targeted therapies are in the pre-clinical phase, except for losmapimod, which is currently undergoing a phase 3 clinical trial.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Given the absence of approved disease-modifying treatments for FSHD, the primary approach for management currently involves multidisciplinary supportive measures which are limited. Recent developments in the form of targeted therapies and strategies for the definitive treatment of FSHD indicate a promising era.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"49 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140566277","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}
Pub Date : 2024-03-26DOI: 10.1007/s11940-024-00788-5
Elana Meer, Michael K. Yoon, Jonathan E. Lu
Purpose of review
This review aims to provide a critical appraisal of current diagnostic and therapeutic strategies for patients with orbital inflammatory disease (OID). We present the reader with a review of clinical, imaging, laboratory, and biopsy based assessment of OID and review the current treatment modalities utilized including corticosteroids, corticosteroid-sparing (immunomodulatory) agents, radiation, antibiotics, and disease specific therapy.
Recent findings
Two major developments and trends have emerged in the management of orbital inflammation. First, improved understanding and distinction of inflammation subtypes (myositis, dacryoadenitis, or infiltrative) allows for more nuanced workup and treatment. Second, immunomodulatory agents have shown promise in achieving disease control in cases of truly idiopathic or corticosteroid-resistant OID. Together, these advances have led to fewer adverse effects and better efficacy.
Summary
The optimal treatment of OID depends on distinguishing between nonspecific and specific inflammation. Nonspecific inflammation tends to respond to corticosteroid therapy with a lower chance of relapse, while specific orbital inflammation often requires targeting the underlying disease with steroid-sparing therapy and immunomodulatory agents.
综述目的 本综述旨在对眼眶炎症性疾病(OID)患者目前的诊断和治疗策略进行批判性评估。我们向读者介绍了眼眶炎症的临床、影像学、实验室和活组织检查评估,并回顾了目前使用的治疗方法,包括皮质类固醇、皮质类固醇辅助(免疫调节)剂、放射线、抗生素和疾病特异性疗法。首先,人们对炎症亚型(肌炎、泪腺炎或浸润性炎症)的认识和区分有所提高,从而可以进行更细致的检查和治疗。其次,免疫调节药物在真正的特发性或皮质类固醇耐药的 OID 病例中有望实现疾病控制。总结OID的最佳治疗取决于区分非特异性炎症和特异性炎症。非特异性炎症往往对皮质类固醇治疗有反应,复发几率较低,而特异性眼眶炎症往往需要针对潜在疾病使用类固醇节省疗法和免疫调节药物。
{"title":"Update on Treatment of Idiopathic (and Non-Idiopathic) Orbital Inflammation","authors":"Elana Meer, Michael K. Yoon, Jonathan E. Lu","doi":"10.1007/s11940-024-00788-5","DOIUrl":"https://doi.org/10.1007/s11940-024-00788-5","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of review</h3><p>This review aims to provide a critical appraisal of current diagnostic and therapeutic strategies for patients with orbital inflammatory disease (OID). We present the reader with a review of clinical, imaging, laboratory, and biopsy based assessment of OID and review the current treatment modalities utilized including corticosteroids, corticosteroid-sparing (immunomodulatory) agents, radiation, antibiotics, and disease specific therapy.</p><h3 data-test=\"abstract-sub-heading\">Recent findings</h3><p>Two major developments and trends have emerged in the management of orbital inflammation. First, improved understanding and distinction of inflammation subtypes (myositis, dacryoadenitis, or infiltrative) allows for more nuanced workup and treatment. Second, immunomodulatory agents have shown promise in achieving disease control in cases of truly idiopathic or corticosteroid-resistant OID. Together, these advances have led to fewer adverse effects and better efficacy.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>The optimal treatment of OID depends on distinguishing between nonspecific and specific inflammation. Nonspecific inflammation tends to respond to corticosteroid therapy with a lower chance of relapse, while specific orbital inflammation often requires targeting the underlying disease with steroid-sparing therapy and immunomodulatory agents.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"154 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140299468","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}
Pub Date : 2024-03-21DOI: 10.1007/s11940-024-00785-8
Ram Mani, Ahmad Almelegy, Thu Minh Truong, Gaurav N. Pathak, Mary L. Wagner, Cindy Wassef
Purpose of review
This review describes risk factors for severe skin reactions to antiseizure medications (ASMs), the usage of updated tests to predict those with increased risk of a severe cutaneous reaction, and guides how to choose specific ASMs and dosing to lower the risk for these reactions. Information is given regarding specific mild versus severe reactions, initial diagnostic evaluation, and treatment. A table listing the risk of mild and severe cutaneous reaction risks as well as the management of potential seizures that may occur while stopping the culprit ASM are provided.
Recent findings
Five new ASMs have joined the total of 26 FDA-approved ASMs since 2018. Cenobamate had three patients develop a drug reaction with eosinophilia and systemic symptoms. A lower starting dosing and slower titration have resulted in no further published cases. Based on limited data, rash risk is low for fenfluramine, ganaxalone, and stiripentol. It is low-moderate for Epidiolex. Molecular tests can predict severe reactions.
Summary
Skin reactions are a relatively common side effect of ASMs with aromatic ASMs having the greatest risk. Identifying and informing high-risk patients when to seek medical attention, stopping the culprit ASM when a severe reaction looks possible, and providing appropriate medical triage can reduce morbidity and mortality from severe skin and systemic reactions.
{"title":"Distinguishing Benign Rashes From Severe Skin Reactions From Anti-Seizure Medications","authors":"Ram Mani, Ahmad Almelegy, Thu Minh Truong, Gaurav N. Pathak, Mary L. Wagner, Cindy Wassef","doi":"10.1007/s11940-024-00785-8","DOIUrl":"https://doi.org/10.1007/s11940-024-00785-8","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of review</h3><p>This review describes risk factors for severe skin reactions to antiseizure medications (ASMs), the usage of updated tests to predict those with increased risk of a severe cutaneous reaction, and guides how to choose specific ASMs and dosing to lower the risk for these reactions. Information is given regarding specific mild versus severe reactions, initial diagnostic evaluation, and treatment. A table listing the risk of mild and severe cutaneous reaction risks as well as the management of potential seizures that may occur while stopping the culprit ASM are provided.</p><h3 data-test=\"abstract-sub-heading\">Recent findings</h3><p>Five new ASMs have joined the total of 26 FDA-approved ASMs since 2018. Cenobamate had three patients develop a drug reaction with eosinophilia and systemic symptoms. A lower starting dosing and slower titration have resulted in no further published cases. Based on limited data, rash risk is low for fenfluramine, ganaxalone, and stiripentol. It is low-moderate for Epidiolex. Molecular tests can predict severe reactions.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Skin reactions are a relatively common side effect of ASMs with aromatic ASMs having the greatest risk. Identifying and informing high-risk patients when to seek medical attention, stopping the culprit ASM when a severe reaction looks possible, and providing appropriate medical triage can reduce morbidity and mortality from severe skin and systemic reactions.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"82 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140202161","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}
Pub Date : 2024-03-21DOI: 10.1007/s11940-024-00787-6
Vasilios C. Constantinides, Nikolaos Giagkou, Maria-Evgenia Brinia, Christos Koros, Leonidas Stefanis, Maria Stamelou
Purpose of Review
Atypical parkinsonism is a term usually used to describe three neurodegenerative parkinsonian disorders: progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA). In contrast to Parkinson’s disease, these disorders have a poor prognosis and present with a multitude of diverse symptoms, including parkinsonism, dystonia, myoclonus, gait disorders, dysarthria, dysphagia, sleep, cognitive, and behavioral disorders. In the absence of an approved disease-modifying treatment, symptomatic treatment is the mainstay of management. The purpose of this review is to present an overview of the management of these complex disorders, with a particular focus on a holistic and multidisciplinary approach.
Recent Findings
In addition to presenting the most significant pharmacological interventions for symptom management, data regarding non-pharmacological interventions are analyzed. Important non-pharmacological clinical practice questions such as breaking the news, palliative care, and end-of-life issues are discussed, in an effort to present an overview of the management of atypical parkinsonism from diagnosis to the most advanced stages of these diseases.
Summary
Management of atypical parkinsonian disorders includes symptomatic pharmacological and non-pharmacological interventions, in addition to addressing issues such as informing the patient of the diagnosis, palliative care, and end-of-life issues, which require a multidisciplinary approach.
{"title":"Management Strategies for Atypical Parkinsonism","authors":"Vasilios C. Constantinides, Nikolaos Giagkou, Maria-Evgenia Brinia, Christos Koros, Leonidas Stefanis, Maria Stamelou","doi":"10.1007/s11940-024-00787-6","DOIUrl":"https://doi.org/10.1007/s11940-024-00787-6","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of Review</h3><p>Atypical parkinsonism is a term usually used to describe three neurodegenerative parkinsonian disorders: progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA). In contrast to Parkinson’s disease, these disorders have a poor prognosis and present with a multitude of diverse symptoms, including parkinsonism, dystonia, myoclonus, gait disorders, dysarthria, dysphagia, sleep, cognitive, and behavioral disorders. In the absence of an approved disease-modifying treatment, symptomatic treatment is the mainstay of management. The purpose of this review is to present an overview of the management of these complex disorders, with a particular focus on a holistic and multidisciplinary approach.</p><h3 data-test=\"abstract-sub-heading\">Recent Findings</h3><p>In addition to presenting the most significant pharmacological interventions for symptom management, data regarding non-pharmacological interventions are analyzed. Important non-pharmacological clinical practice questions such as breaking the news, palliative care, and end-of-life issues are discussed, in an effort to present an overview of the management of atypical parkinsonism from diagnosis to the most advanced stages of these diseases.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Management of atypical parkinsonian disorders includes symptomatic pharmacological and non-pharmacological interventions, in addition to addressing issues such as informing the patient of the diagnosis, palliative care, and end-of-life issues, which require a multidisciplinary approach.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"25 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140201950","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}
Pub Date : 2024-03-21DOI: 10.1007/s11940-024-00786-7
Abstract
Purpose of review
Autoimmune encephalitis (AE) is increasingly recognized as a treatable cause of encephalitis in children. While prior observational studies demonstrate improved motor outcomes with early immunotherapy, less is known about long-term management and treatment for relapsing disease. In this review, we present current treatment approaches to pediatric AE, in particular relapse risk and treatment for relapsing AE in children.
Recent findings
A recent meta-analysis of anti-NMDAR encephalitis demonstrated that disease onset in adolescence was associated with an increased odds of relapse whereas treatment with rituximab and IVIG for 6 months or longer were associated with a non-relapsing course. However, no specific pediatric sub-analyses were reported. A single-center study on adult and pediatric AE showed that rituximab use was associated with a reduction in time to relapse and recurring relapses although the data for the pediatric cohort did not achieve statistical significance.
Summary
The use of second-line immunotherapy during the initial attack may reduce the risk for relapsing disease in pediatric AE. Larger studies are needed to investigate relapse risk and treatment in both anti-NMDAR and non-NMDAR encephalitis in children.
{"title":"Treatment Approaches in Pediatric Relapsing Autoimmune Encephalitis","authors":"","doi":"10.1007/s11940-024-00786-7","DOIUrl":"https://doi.org/10.1007/s11940-024-00786-7","url":null,"abstract":"<h3>Abstract</h3> <span> <h3>Purpose of review</h3> <p>Autoimmune encephalitis (AE) is increasingly recognized as a treatable cause of encephalitis in children. While prior observational studies demonstrate improved motor outcomes with early immunotherapy, less is known about long-term management and treatment for relapsing disease. In this review, we present current treatment approaches to pediatric AE, in particular relapse risk and treatment for relapsing AE in children.</p> </span> <span> <h3>Recent findings</h3> <p>A recent meta-analysis of anti-NMDAR encephalitis demonstrated that disease onset in adolescence was associated with an increased odds of relapse whereas treatment with rituximab and IVIG for 6 months or longer were associated with a non-relapsing course. However, no specific pediatric sub-analyses were reported. A single-center study on adult and pediatric AE showed that rituximab use was associated with a reduction in time to relapse and recurring relapses although the data for the pediatric cohort did not achieve statistical significance.</p> </span> <span> <h3>Summary</h3> <p>The use of second-line immunotherapy during the initial attack may reduce the risk for relapsing disease in pediatric AE. Larger studies are needed to investigate relapse risk and treatment in both anti-NMDAR and non-NMDAR encephalitis in children.</p> </span>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"12 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140201959","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}
Pub Date : 2024-03-15DOI: 10.1007/s11940-024-00789-4
Nathan A. Shlobin
Purpose of Review
To care for patients with cerebrovascular disease (CVD), neurointerventionalists, intensivists, and other healthcare providers must be equipped to address associated ethical challenges. This review aims to delineate the applicability of fundamental bioethical approaches to CVD, highlight key ethical issues in CVD care, and delineate an ethical framework to streamline ethical decision-making for people with CVD.
Recent Findings
Three introductory cases are presented. The four key principles of principalism and the approach of narrative ethics are described with reference to CVD. Key ethical considerations include decision-making capacity and informed consent, uncertainty, and resource allocation. A categorization of CVD as emergent/nonemergent and the recommended management as intervention/no intervention helps frame the spectrum of CVD. A different six-pathway may then be taken based on which category the patient case corresponds to.
Summary
Physicians involved in the care of people with cerebrovascular disease must understand how the ethical issues manifest in individual patient cases to ensure appropriate care. The aforementioned ethical framework may aid physicians in providing ethically sound care. All decisions must involve a balance between clinical expertise and patient values and preferences or those articulated by a surrogate to properly respect the wishes of patients with CVD.
{"title":"Ethical Considerations in the Treatment of Cerebrovascular Disease","authors":"Nathan A. Shlobin","doi":"10.1007/s11940-024-00789-4","DOIUrl":"https://doi.org/10.1007/s11940-024-00789-4","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose of Review</h3><p>To care for patients with cerebrovascular disease (CVD), neurointerventionalists, intensivists, and other healthcare providers must be equipped to address associated ethical challenges. This review aims to delineate the applicability of fundamental bioethical approaches to CVD, highlight key ethical issues in CVD care, and delineate an ethical framework to streamline ethical decision-making for people with CVD.</p><h3 data-test=\"abstract-sub-heading\">Recent Findings</h3><p>Three introductory cases are presented. The four key principles of principalism and the approach of narrative ethics are described with reference to CVD. Key ethical considerations include decision-making capacity and informed consent, uncertainty, and resource allocation. A categorization of CVD as emergent/nonemergent and the recommended management as intervention/no intervention helps frame the spectrum of CVD. A different six-pathway may then be taken based on which category the patient case corresponds to.</p><h3 data-test=\"abstract-sub-heading\">Summary</h3><p>Physicians involved in the care of people with cerebrovascular disease must understand how the ethical issues manifest in individual patient cases to ensure appropriate care. The aforementioned ethical framework may aid physicians in providing ethically sound care. All decisions must involve a balance between clinical expertise and patient values and preferences or those articulated by a surrogate to properly respect the wishes of patients with CVD.</p>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"25 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140151826","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}
Pub Date : 2024-02-06DOI: 10.1007/s11940-023-00779-y
Kristen Nobles, Kiersten Norby, Kristina Small, Monisha A. Kumar
Purpose of Review
Bed rest was a treatment recommended for critically ill patients admitted to the intensive care unit (ICU) that aimed to minimize energy expenditure, permit wound healing and minimize somatic stressors. However, evidence demonstrates that bed rest leads to disuse atrophy, which may be compounded by premorbid sarcopenia and ICU-acquired weakness (ICUAW). ICUAW partly results from muscle breakdown and systemic inflammation and may exacerbate critical illness. Coupled with analgosedation, ICUAW may prolong mechanical ventilation (Kho et al. in BMJ Open Respir Res. 2019;6(1) 2019; Maffei et al. in Arch Phys Med Rehabil. 2017;98 2017; McWilliams et al. in J Crit Care. 2018;44 2018; Sarfati et al. in J Crit Care. 2018;46 2018), increase risk of venous thromboembolism (Denehy et al. in Intensive Care Med. 2017;43(1) 2017; Lyles in J Am Geriatr Soc. 1988;36(11) 1988) create dependence on vasopressor agents (Lyles in J Am Geriatr Soc. 1988;36(11) 1988; Fortney et al. in Comprehen Physiol. 1996) restrict joint mobility, and induce pressure injuries. Neurologically injured patients may be at a higher risk of ICUAW than other critically-ill patients, given that neurological injury itself results in weakness, which may be focal or generalized. Early mobilization (EM), typically defined as physical therapy within 72 h of ICU (Cumming et al. in Neurology. 2019;93(7) 2019), may preempt or mitigate these deleterious consequences of critical care.
Recent Findings
Retrospective data suggest that EM protocols reduce ventilator days, decrease venous thromboembolism, avert pressure wounds, and reduce the length of stay. EM may reduce the incidence, duration, and severity of delirium (Morris et al. in Crit Care Med. 2008;36(8) 2008; Needham et al. in Arch Phys Med Rehabil. 2010;91(4) 2010). Larger and more rigorous studies have not demonstrated benefit of EM on outcomes after critical care; some have demonstrated harm. Neurologically injured critical care patients have generally been excluded from early mobilization protocols due to safety concerns that stem from the increased potential for falls, disorders of consciousness, cognitive impairment, intracranial hypertension, and potential dislodgment of intracranial devices. Notably, data from patients with ischemic stroke suggest that EM may also be associated with harm in this group.
Summary
EM may benefit neurologically injured patients once acute ischemia, elevated ICP, and seizures are resolved. Targeting moderate acuity patients may be critical to improving outcomes and optimizing resource utilization in this resource-intensive intervention. The duration of mobility session, optimal frequency of mobility session, and timing of session remain to be determined.
综述目的卧床休息是重症监护室(ICU)建议对重症患者采取的一种治疗方法,旨在最大限度地减少能量消耗、促进伤口愈合并减少躯体压力。然而,有证据表明,卧床休息会导致废用性萎缩,而病前肌少症和重症监护室获得性乏力(ICUAW)可能会加重这种情况。ICUAW 的部分原因是肌肉分解和全身炎症,可能会加重危重病人的病情。加上镇痛,ICUAW 可能会延长机械通气时间(Kho 等人,发表于 BMJ Open Respir Res. 2019;6(1) 2019;Maffei 等人,发表于 Arch Phys Med Rehabil.2017;98 2017; McWilliams et al. in J Crit Care.2018;44 2018; Sarfati et al. in J Crit Care.2018;46 2018),增加静脉血栓栓塞的风险(Denehy 等,载于 Intensive Care Med.2017;43(1)2017;Lyles 发表于 J Am Geriatr Soc. 1988;36(11)1988),造成对血管加压药的依赖(Lyles 发表于 J Am Geriatr Soc. 1988;36(11)1988;Fortney 等人发表于 Comprehen Physiol.由于神经损伤本身会导致虚弱,而虚弱可能是局灶性的,也可能是全身性的,因此神经损伤患者发生 ICUAW 的风险可能高于其他重症患者。早期动员(EM)通常是指在进入 ICU 72 小时内进行物理治疗(Cumming 等人,发表于 Neurology.最近的研究结果回顾性数据表明,EM 方案可减少呼吸机天数、减少静脉血栓栓塞、避免压伤并缩短住院时间。急救可降低谵妄的发生率、持续时间和严重程度(Morris 等,发表于《重症监护医学》(Crit Care Med.2008;36(8)2008;Needham 等人在 Arch Phys Med Rehabil.2010;91(4) 2010).更大规模和更严格的研究并未证明电磁疗法对危重症护理后的预后有益;有些研究则证明电磁疗法有害。神经系统受伤的危重症患者通常被排除在早期移动方案之外,这是因为跌倒、意识障碍、认知障碍、颅内高压和颅内装置可能脱落的可能性增加,因而存在安全隐患。值得注意的是,来自缺血性中风患者的数据表明,EM 也可能会对这部分患者造成伤害。摘要一旦急性缺血、ICP 升高和癫痫发作得到缓解,EM 可能会使神经损伤患者受益。在这种资源密集型干预中,针对中等严重程度的患者可能是改善预后和优化资源利用的关键。活动治疗的持续时间、活动治疗的最佳频率以及治疗时机仍有待确定。
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Pub Date : 2024-02-01DOI: 10.1007/s11940-024-00780-z
Abstract
Purpose of Review
The purpose of this review is to summarize currently available and developing diagnostic and treatment options for hereditary transthyretin amyloid polyneuropathy. Transthyretin amyloidosis (ATTR) predominantly manifests with cardiomyopathy and/or peripheral neuropathy, but amyloid deposits may be found in other organs or tissues.
Recent Findings
Currently available treatments include transthyretin gene silencers (for hereditary ATTR peripheral neuropathy only) and transthyretin stabilizers (tafamidis for ATTR cardiomyopathy in the USA, and for both hereditary ATTR peripheral neuropathy and ATTR cardiomyopathy in Europe, Japan, Brazil, and some other countries), and liver transplantation. Gene silencers stop the progression of hereditary ATTR peripheral neuropathy in most patients, and transthyretin stabilizers reduce hospitalizations and mortality in patients with ATTR cardiomyopathy. The use of liver transplantation for ATTR has declined with the availability of more effective therapies, and shortage of available allografts. On the horizon are new treatments already in clinical trials including new gene silencers and gene editing agents, new transthyretin stabilizers, and amyloid removal treatments.
Summary
Recently approved treatments for ATTR have changed its natural history, and additional medications may get approved in the near future. Early diagnosis is still essential to improve treatment outcomes. New management strategies may include combinations of gene silencers, transthyretin stabilizers, gene editing, and amyloid removal agents, but the cost may become the limiting factor.
{"title":"Update on Amyloid Polyneuropathy and Treatment","authors":"","doi":"10.1007/s11940-024-00780-z","DOIUrl":"https://doi.org/10.1007/s11940-024-00780-z","url":null,"abstract":"<h3>Abstract</h3> <span> <h3>Purpose of Review</h3> <p>The purpose of this review is to summarize currently available and developing diagnostic and treatment options for hereditary transthyretin amyloid polyneuropathy. Transthyretin amyloidosis (ATTR) predominantly manifests with cardiomyopathy and/or peripheral neuropathy, but amyloid deposits may be found in other organs or tissues.</p> </span> <span> <h3>Recent Findings</h3> <p>Currently available treatments include transthyretin gene silencers (for hereditary ATTR peripheral neuropathy only) and transthyretin stabilizers (tafamidis for ATTR cardiomyopathy in the USA, and for both hereditary ATTR peripheral neuropathy and ATTR cardiomyopathy in Europe, Japan, Brazil, and some other countries), and liver transplantation. Gene silencers stop the progression of hereditary ATTR peripheral neuropathy in most patients, and transthyretin stabilizers reduce hospitalizations and mortality in patients with ATTR cardiomyopathy. The use of liver transplantation for ATTR has declined with the availability of more effective therapies, and shortage of available allografts. On the horizon are new treatments already in clinical trials including new gene silencers and gene editing agents, new transthyretin stabilizers, and amyloid removal treatments.</p> </span> <span> <h3>Summary</h3> <p>Recently approved treatments for ATTR have changed its natural history, and additional medications may get approved in the near future. Early diagnosis is still essential to improve treatment outcomes. New management strategies may include combinations of gene silencers, transthyretin stabilizers, gene editing, and amyloid removal agents, but the cost may become the limiting factor.</p> </span>","PeriodicalId":10975,"journal":{"name":"Current Treatment Options in Neurology","volume":"3 1","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139662289","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}