PURPOSE OF REVIEW: Migraine affects a large portion of the world's population. Migraine encompasses a broad range of symptoms, with broad reaching ramifications in the form of Health-Related Quality of Life (HRQoL) facto...PURPOSE OF REVIEW: Migraine affects a large portion of the world's population. Migraine encompasses a broad range of symptoms, with broad reaching ramifications in the form of Health-Related Quality of Life (HRQoL) factors. In our review we sought to understand the aspects encompassing the burden of disease on both an individual and population level. Furthermore, we reviewed the development and incorporation of Patient Reported Outcome Measures (PROM), questionnaires that assess HRQoL in real time, in how they have been incorporated in clinical research up to now and how they can be utilized in clinical practice moving forward. RECENT FINDINGS: It has been shown that there is much heterogeneity within the field in PROM development processes as well as their utilization in episodic migraine (EM) clinical trials. Furthermore, they are inconsistently used in clinical practice. Among the most commonly used PROMs, the MSQv2.1 is among the most valid and reliable. Beyond that, it also shows promise to help in guidance of clinical management of migraine.
Curr Neurol Neurosci Rep
· 2024 Nov · PMID 39304580
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PURPOSE OF REVIEW: Sickle cell anemia (SCA) is an autosomal recessive inherited hemoglobinopathy that results in a high risk of stroke. SCA primarily affects an underserved minority population of children who are frequen...PURPOSE OF REVIEW: Sickle cell anemia (SCA) is an autosomal recessive inherited hemoglobinopathy that results in a high risk of stroke. SCA primarily affects an underserved minority population of children who are frequently not receiving effective, multi-disciplinary, preventative care. This article reviews primary and secondary stroke prevention and treatment for children with SCA for the general adult and pediatric neurologist, who may play an important role in providing critical neurologic evaluation and care to these children. RECENT FINDINGS: Primary stroke prevention is efficacious at reducing ischemic stroke risk, but it is not consistently implemented into clinical practice in the United States, resulting in these children remaining at high risk. Acute symptomatic stroke management requires neurology involvement and emergent transfusion to limit ischemia. Furthermore, while chronic transfusion therapy is a proven secondary preventative modality for those with prior symptomatic or silent cerebral infarcts, it carries significant burden. Newer therapies (e.g., stem cell therapies and voxelotor) deserve further study as they may hold promise in reducing stroke risk and treatment burden. Effective primary and secondary stroke prevention and treatment remain a challenge. Informing and engaging neurology providers to recognize and provide critical neurologic evaluation and treatment has potential to close care gaps.
PURPOSE OF REVIEW: Sleep disturbances are a hallmark feature of various autoimmune neurological diseases (AINDs). However, limited awareness of these sleep manifestations exists among clinicians. We provide a comprehensi...PURPOSE OF REVIEW: Sleep disturbances are a hallmark feature of various autoimmune neurological diseases (AINDs). However, limited awareness of these sleep manifestations exists among clinicians. We provide a comprehensive overview of assessment methods, characteristic sleep disturbances, the impact of specific antibodies on sleep patterns, and treatment strategies for sleep disturbances in AINDs. RECENT FINDINGS: Research advancements in sleep disturbances in autoimmune neurological disease focus primarily on four areas: mechanisms, clinical characteristics, assessment, and treatment. Regarding mechanisms, animal models for AINDs, particularly those involving specific antibodies like anti-NMDAR, anti-LGI1, and anti-IgLON5, have become more comprehensive. Recent advancements in animal models have led to the establishment of numerous models for AINDs; these models include a wide range of antibodies, including anti-NMDAR, anti-LGI1, and anti-IgLON5. Several studies using these models have revealed common mechanisms underlying sleep disturbances in these diseases. In terms of clinical characteristics, the identification of antibodies associated with recently discovered AINDs has expanded the spectrum of sleep disturbance symptoms observed compared to prior findings. A comprehensive evaluation system for the assessment of sleep disturbances has been established, including questionnaires, polysomnography, functional magnetic resonance imaging, and F-FDG PET/CT. Additionally, cardiopulmonary coupling shows promise as a novel assessment tool. Currently, no universally effective treatment exists for sleep disturbances in autoimmune neurological diseases, either through symptomatic treatment or immunosuppressive therapy. Further studies are needed to confirm the efficacy of new therapies and validate the benefits of existing treatments. Sleep disturbances are a hallmark feature of AINDs. Recent advancements have significantly expanded our understanding of their assessment and treatment. However, further studies are needed to address the remaining uncertainties in sleep disturbance management.
PURPOSE OF REVIEW: The identification of isocitrate dehydrogenase (IDH) mutations has led to a transformation in our understanding of gliomas and has paved the way to a new era of targeted therapy. In this article, we re...PURPOSE OF REVIEW: The identification of isocitrate dehydrogenase (IDH) mutations has led to a transformation in our understanding of gliomas and has paved the way to a new era of targeted therapy. In this article, we review the classification of IDH-mutant glioma, standard of care treatment options, clinical evidence for mutant IDH (mIDH) inhibitors, and practical implications of the recent landmark INDIGO trial. RECENT FINDINGS: In the phase 3 randomized placebo-controlled INDIGO trial, mIDH1/2 inhibitor vorasidenib increased progression-free survival among non-enhancing grade 2 IDH-mutant gliomas following surgery. This marks the first positive randomized trial of targeted therapy in IDH-mutant glioma, and led to the US Food and Drug Administration's approval of vorasidenib in August 2024 for grade 2 IDH-mutant glioma. Vorasidenib is a well-tolerated treatment that can benefit a subset of patients with IDH-mutant glioma. Targeting mIDH also remains a promising strategy for select groups of patients excluded from the INDIGO trial. Ongoing and future studies, including with new agents and with combination therapy approaches, may expand the benefit and unlock the potential of mIDH inhibitors.
Curr Neurol Neurosci Rep
· 2024 Dec · PMID 39266871
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PURPOSE OF REVIEW: Population receptive field (pRF) modeling is an fMRI technique used to retinotopically map visual cortex, with pRF size characterizing the degree of spatial integration. In clinical populations, most p...PURPOSE OF REVIEW: Population receptive field (pRF) modeling is an fMRI technique used to retinotopically map visual cortex, with pRF size characterizing the degree of spatial integration. In clinical populations, most pRF mapping research has focused on damage to visual system inputs. Herein, we highlight recent work using pRF modeling to study high-level visual dysfunctions. RECENT FINDINGS: Larger pRF sizes, indicating coarser spatial processing, were observed in homonymous visual field deficits, aging, and autism spectrum disorder. Smaller pRF sizes, indicating finer processing, were observed in Alzheimer's disease and schizophrenia. In posterior cortical atrophy, a unique pattern was found in which pRF size changes depended on eccentricity. Changes to pRF properties were observed in clinical populations, even in high-order impairments, explaining visual behavior. These pRF changes likely stem from altered interactions between brain regions. Furthermore, some studies suggested that pRF sizes change as part of cortical reorganization, and they can point towards future prognosis.
Wu HC, Gombolay GY, Yang JH
… +3 more, Graves JS, Christy A, Xiang XM
Curr Neurol Neurosci Rep
· 2024 Oct · PMID 39259430
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PURPOSE OF REVIEW: B-cell depletion therapy, including anti-CD20 and anti-CD19 therapies, is increasingly used for a variety of autoimmune and conditions, including those affecting the central nervous system. However, B-...PURPOSE OF REVIEW: B-cell depletion therapy, including anti-CD20 and anti-CD19 therapies, is increasingly used for a variety of autoimmune and conditions, including those affecting the central nervous system. However, B-cell depletion therapy use can be complicated by adverse effects associated with administration and immunosuppression. This review aims to summarize the application of anti-CD20 and anti-CD19 therapies for the pediatric neurologist and neuroimmunologist. RECENT FINDINGS: Most existing literature come from clinical trials with adult patients, although more recent studies are now capturing the effects of these therapies in children. The most common side effects include infusion related reactions and increased infection risk from immunosuppression. Several strategies can mitigate infusion related reactions. Increased infections due to persistent hypogammaglobulinemia can benefit from replacement immunoglobulin. B-cell depletion therapies can be safe and effective in pediatric patients. Anticipation and mitigation of common adverse effects through primary prevention strategies, close monitoring, and appropriate symptomatic management can improve safety and tolerability.
PURPOSE OF REVIEW: This review aims to rediscuss the leading theories concerning the role of basal ganglia and the thalamus in the genesis of aphasic symptoms in the absence of gross anatomical lesions in cortical langua...PURPOSE OF REVIEW: This review aims to rediscuss the leading theories concerning the role of basal ganglia and the thalamus in the genesis of aphasic symptoms in the absence of gross anatomical lesions in cortical language areas as assessed by conventional neuroimaging studies. RECENT FINDINGS: New concepts in language processing and modern neuroimaging techniques have enabled some progress in resolving the impasse between the current dominant theories: (a) direct and specific linguistic processing and (b) subcortical structures as processing relays in domain-general functions. Of particular interest are studies of connectivity based on functional magnetic resonance imaging (MRI) and tractography that highlight the impact of white matter pathway lesions on aphasia development and recovery. Connectivity studies have put into evidence the central role of the arcuate fasciculus (AF), inferior frontal occipital fasciculus (IFOF), and uncinate fasciculus (UF) in the genesis of aphasia. Regarding the thalamus, its involvement in lexical-semantic processing through modulation of the frontal cortex is becoming increasingly apparent.
PURPOSE OF REVIEW: Patients with various neurological disorders often present with or manifest during their disease process laryngologic pathology that can lead to significant morbidity. Recognizing and treating this asp...PURPOSE OF REVIEW: Patients with various neurological disorders often present with or manifest during their disease process laryngologic pathology that can lead to significant morbidity. Recognizing and treating this aspect of their disease may be crucial in optimizing patient outcome. RECENT FINDINGS: We discuss updated information and management regarding various neurological disorders that affect the larynx and how these sequelae are diagnosed and treated. An understanding of the laryngologic manifestations of neurological disorders will facilitate management of these patient populations. Preventing and minimizing complications arising from these sequelae will improve quality of life and optimize patient outcomes.
PURPOSE OF REVIEW: Misdiagnosis of multiple sclerosis (MS) is a prevalent worldwide problem. This review discusses how MS misdiagnosis has evolved over time and focuses on contemporary challenges and potential strategies...PURPOSE OF REVIEW: Misdiagnosis of multiple sclerosis (MS) is a prevalent worldwide problem. This review discusses how MS misdiagnosis has evolved over time and focuses on contemporary challenges and potential strategies for its prevention. RECENT FINDINGS: Recent studies report cohorts with a range of misdiagnosis between 5 and 18%. Common disorders are frequently misdiagnosed as MS. Overreliance on MRI findings and misapplication of MS diagnostic criteria are often associated with misdiagnosis. Emerging imaging biomarkers, including the central vein sign and paramagnetic rim lesions, may aid diagnostic accuracy when evaluating patients for suspected MS. MS misdiagnosis can have harmful consequences for patients and healthcare systems. Further research is needed to better understand its causes. Concerted and novel educational efforts to ensure accurate and widespread implementation of MS diagnostic criteria remain an unmet need. The incorporation of diagnostic biomarkers highly specific for MS in the future may prevent misdiagnosis.
PURPOSE OF REVIEW: The overwhelming majority of stroke burden can be prevented through the pillars of lifestyle medicine: diet, exercise, sleep, substance abuse, stress management, and healthy relationships. Among these,...PURPOSE OF REVIEW: The overwhelming majority of stroke burden can be prevented through the pillars of lifestyle medicine: diet, exercise, sleep, substance abuse, stress management, and healthy relationships. Among these, diet confers the greatest attributable risk. RECENT FINDINGS: Despite abundant data and integration of lifestyle medicine within major stroke prevention guidelines, several barriers to effective implementation remain. These include lack of emphasis in medical education, integration in hospital certification metrics, reimbursement from medical insurance, and health policy that inadequately addresses social determinants of health. However, both top-down and bottom-up solutions introduced within the last few years are helping to break down these barriers. This review highlights recent literature and interventions that are closing the gap between the theory and practice of stroke prevention through lifestyle risk factors from a US perspective. By strategically targeting the various institutional barriers, it is possible and essential to substantially reduce stroke burden.
PURPOSE OF REVIEW: We aim to critically review animal and human studies of the glymphatic system in migraine and propose a model for how the glymphatic system may function in migraine, based on the available evidence. RE...PURPOSE OF REVIEW: We aim to critically review animal and human studies of the glymphatic system in migraine and propose a model for how the glymphatic system may function in migraine, based on the available evidence. RECENT FINDINGS: Early studies in animal models report migraine attacks temporarily disrupt glymphatic flow. Human imaging studies suggest chronic migraine may be associated with alterations in glymphatic system function, albeit with conflicting results. Presently, it remains unknown whether repetitive migraine attacks or frequent nights of insomnia impair glymphatic system function over time in those with migraine, and whether alterations in glymphatic function could contribute to worsening migraine disability or risk for cognitive disease. Longitudinal studies of glymphatic function in patients with migraine and insomnia, with inclusion of cognitive assessments, may be informative.
Curr Neurol Neurosci Rep
· 2024 Oct · PMID 39150649
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PURPOSE OF REVIEW: Recent stroke treatment advances have necessitated agile, broad-scale healthcare system redesign, to achieve optimal patient outcomes and access equity. Optimised hyperacute stroke care requires integr...PURPOSE OF REVIEW: Recent stroke treatment advances have necessitated agile, broad-scale healthcare system redesign, to achieve optimal patient outcomes and access equity. Optimised hyperacute stroke care requires integrated pre-hospital, emergency department, stroke specialist, radiology, neurosurgical and endovascular neurointervention services, guided by a population-wide needs analysis. In this review, we survey system integration efforts, providing case studies, and identify common elements of successful initiatives. RECENT FINDINGS: Different regions and nations have evolved varied acute stroke systems depending on geography, population density and workforce. However, common facilitators to these solutions have included stroke unit care as a foundation, government-clinician synergy, pre-hospital pathway coordination, service centralisation, and stroke data guiding system improvement. Further technological advantages will minimize the geographical distance disadvantages and facilitate virtual expertise redistribution to remote areas. Continued treatment advances necessitate an integrated, adaptable, population-wide trans-disciplinary approach. A well-designed clinician-led and government-supported system can facilitate hyperacute care and scaffold future system enhancements.
PURPOSE OF REVIEW: Pediatric low-grade gliomas (pLGGs) often result in significant long-term morbidities despite high overall survival rates. This review aims to consolidate the current understanding of pLGG biology and...PURPOSE OF REVIEW: Pediatric low-grade gliomas (pLGGs) often result in significant long-term morbidities despite high overall survival rates. This review aims to consolidate the current understanding of pLGG biology and molecular features and provide an overview of current and emerging treatment strategies. RECENT FINDINGS: Surgical resection remains a primary treatment modality, supplemented by chemotherapy and radiotherapy in specific cases. However, recent advances have elucidated the molecular underpinnings of pLGGs, revealing key genetic abnormalities such as BRAF fusions and mutations and the involvement of the RAS/MAPK and mTOR pathways. Novel targeted therapies, including MEK, BRAF and pan-RAF inhibitors, have shown promise in clinical trials, demonstrating significant efficacy and manageable toxicity. Understanding of pLGGs has significantly improved, leading to more personalized treatment approaches. Targeted therapies have emerged as effective alternatives, potentially reducing long-term toxicities. Future research should focus on optimizing therapy sequences, understanding long-term impacts, and ensuring global accessibility to advanced treatments.
PURPOSE OF REVIEW: Intracerebral hemorrhage (ICH) is the most devastating type of stroke, causing widespread disability and mortality. Unfortunately, the acute care of ICH has lagged behind that of ischemic stroke. There...PURPOSE OF REVIEW: Intracerebral hemorrhage (ICH) is the most devastating type of stroke, causing widespread disability and mortality. Unfortunately, the acute care of ICH has lagged behind that of ischemic stroke. There is an increasing body of evidence supporting the importance of early interventions including aggressive control of blood pressure and reversal of anticoagulation in the initial minutes to hours of presentation. This review highlights scientific evidence behind a new paradigm to care for these patients called Code-ICH. RECENT FINDINGS: While numerous trials aimed at decreasing hematoma expansion through single interventions had failed to show statistically significant effects on primary outcomes, time-sensitive, multifaceted, bundled care approaches have recently shown substantial promise in improving functional outcomes in patients with ICH. The concept of Code-ICH can serve as a structural platform for the practice of acute care neurology to continuously measure its performance, reflect on best practices, advance care, and address disparities.
PURPOSE OF REVIEW: The current review aims to provide an overview of migraine treatment strategies in medically complex patients, including those with renal, liver, and cardiovascular disease. RECENT FINDINGS: In cardiov...PURPOSE OF REVIEW: The current review aims to provide an overview of migraine treatment strategies in medically complex patients, including those with renal, liver, and cardiovascular disease. RECENT FINDINGS: In cardiovascular disease, gepants are likely safe for acute therapy; NSAIDs, ergotamines, and triptans are not recommended. Beta-blockers, ACEi/ARBs, and verapamil have potential cardiovascular benefits in addition to migraine preventive benefit. Frovatriptan requires no dose adjustments in kidney disease or in mild to moderate liver disease. Gepants are safe acute and preventive treatment options in mild and moderate renal and hepatic disease. TCAs and valproic acid require no dose adjustments in renal disease. OnabotulinumtoxinA is likely safe in cardiac, renal, and hepatic impairment. Although CGRP monoclonal antibodies are likely safe in renal and hepatic disease, further study is needed in these conditions as well as in cardiac disease, and no dosing recommendations are available. Effective options are available for those with complex medical comorbidities. Further research is required on the safety of newer migraine-specific therapies in these complex populations.
Piramide N, De Micco R, Siciliano M
… +2 more, Silvestro M, Tessitore A
Curr Neurol Neurosci Rep
· 2024 Oct · PMID 39046642
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PURPOSE OF THE REVIEW: In this review, we attempt to summarize the most updated studies that applied resting-state functional magnetic resonance imaging (rs-fMRI) in the field of Parkinsonisms and related dementia. RECEN...PURPOSE OF THE REVIEW: In this review, we attempt to summarize the most updated studies that applied resting-state functional magnetic resonance imaging (rs-fMRI) in the field of Parkinsonisms and related dementia. RECENT FINDINGS: Over the past decades, increasing interest has emerged on investigating the presence and pathophysiology of cognitive symptoms in Parkinsonisms and their possible role as predictive biomarkers of neurodegenerative brain processes. In recent years, evidence has been provided, applying mainly three methodological approaches (i.e. seed-based, network-based and graph-analysis) on rs-fMRI data, with promising results. Neural correlates of cognitive impairment and dementia have been detected in patients with Parkinsonisms along the diseases course. Interestingly, early functional connectivity signatures were proposed to track and predict future progression of neurodegenerative processes. However, longitudinal studies are still sparce and further investigations are needed to overcome this knowledge gap.
PURPOSE: This paper describes a new surgical procedure with electrical stimulation of the facial nerve for unresolved Bell's palsy and compares the facial nerve recovery with another group who underwent traditional middl...PURPOSE: This paper describes a new surgical procedure with electrical stimulation of the facial nerve for unresolved Bell's palsy and compares the facial nerve recovery with another group who underwent traditional middle cranial fossa decompression. RECENT FINDINGS: All patients with total unilateral facial paralysis had surgery by the senior author 3 months from onset of Bell's Palsy. Surgical decompression was performed in 13 patients between 1992-2012 (Group 1). Surgical exposure with intraoperative electrical stimulation of the facial nerve in the peri-geniculate region was performed in 47 patients between 2012-2022 (Group 2). The facial recovery at 1 month and 3 month were significantly better in Group 2. The degree of synkinesis was significantly less in Group 2. The trans-mastoid electrical stimulation of the facial nerve is less invasive, requires no hospital stay, and less time off work compared to the middle cranial fossa approach. The earlier facial movement at one month results in less long-term unwanted faulty regeneration or synkinesis.
Raheel K, See QR, Munday V
… +11 more, Fakhroo B, Ivanenko O, Salvatelli ML, Mutti C, Goadsby PJ, Delogu A, Naismith SL, Holland P, Parrino L, Chaudhuri KR, Rosenzweig I
Curr Neurol Neurosci Rep
· 2024 Sep · PMID 39031323
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PURPOSE OF REVIEW: Sleep disturbances are amongst most frequent non-motor symptoms of Parkinson's Disease (PD), and they are similarly frequently reported in other alpha-syncleinopathies, such as Dementia with Lewy Bodie...PURPOSE OF REVIEW: Sleep disturbances are amongst most frequent non-motor symptoms of Parkinson's Disease (PD), and they are similarly frequently reported in other alpha-syncleinopathies, such as Dementia with Lewy Bodies (DLB) and Multiple System Atrophy (MSA). More recently, the orexin system has been implicated in control of arousal based on salient environmental set points, and its dysregulation in sleep issues in alpha-synucleinopathies suggested by the findings from the translational animal models. However, its role in the patients with alpha-synucleinopathies remains unclear. We thus set to systematically review, and to critically assess, contemporary evidence on the association of the orexinergic system and sleep disturbances in alpha-synucleinopathies. In this systematic review, studies investigating orexin and sleep in alpha-synucleinopathies (Rapid Eye Movement (REM) Behaviour Disorder (RBD), Parkinson's Disease (PD), Dementia with Lewy Bodies (DLB), Multiple System Atrophy (MSA)) were identified using electronic database searches of PubMed, Web of Science and PsychINFO using MeSH terms, keywords, and title words such as "Alpha-synucleinopathies" AND "Orexin" AND "Sleep Disturbances". RECENT FINDINGS: 17 studies were included in this systemic review, of which 2 studies on RBD, 10 on PD, 4 on DLB, and 1 on MSA patients. Taken together, RBD and PD studies suggest a potential adaptive increase in orexin levels in early stages of the neurodegenerative process, with reduced levels more often reported for later, more advanced stages of illness. To date, no differences in orexin levels were demonstrated between MSA patients and healthy controls. There is a dearth of studies on the role of orexin levels in alpha-synucleinopathies. Moreover, significant methodologic limitations in the current body of work, including use of non-standardised research protocols and lack of prospective, multi-centre studies, disallow for any finite conclusion in regards to underlying pathomechanisms. Nonetheless, a picture of a complex, multifaceted relationship between the dysregulation of the orexinergic pathway and sleep disturbances in alpha-synucleinopathies is emerging. Hence, future studies disentangling orexinergic pathomechanisms of alpha-syncleinopathies are urgently needed to obtain a more comprehensive account of the role of orexinergic pathway in alpha-synucleinopathies. Pharmacological manipulations of orexins may have multiple therapeutic applications in treatment strategies, disease diagnosis, and might be effective for treating both motor and non-motor symptoms.