Current Neurology and Neuroscience Reports

Wolf, Colten; Elahi, Behzad; Charous, Steven

doi: 10.1007/s11910-024-01374-7pmid: 39254875

Purpose of ReviewPatients 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 FindingsWe discuss updated information and management regarding various neurological disorders that affect the larynx and how these sequelae are diagnosed and treated.SummaryAn 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.

Sen, Ansu; Mukherjee, Angshuman; Chakravarty, Ambar

doi: 10.1007/s11910-024-01381-8pmid: 39432226

Purpose of ReviewTo describe different pitfalls in the diagnosis of primary cluster headaches (CHs) with the guidance of seven case vignettes.Recent FindingsThe question of whether primary CHs and migraines are totally different entities has been long debated. Autonomic features can be detected in as many as 60% of migraine patients. Although some genetic similarities have been found, CACNA1A mutations have not been detected among CH patients with hemimotor aura in contrast to hemiplegic migraine. Recently, functional MRI studies have shown that the left thalamic network was the most discriminative MRI feature in distinguishing migraine from CH patients. Compared to migraine, CH patients showed decreased functional interaction between the left thalamus and cortical areas mediating interception and sensory integration. However, clinically the most significant feature had been the restlessness and agitation seen during headache attacks patients with CHs. This feature is also important in distinguishing cluster patients from other patients having other trigeminal autonomic cephalalgias except for a subset of patients with hemicrania continua.SummaryCH is an important member of the group of headache disorders characterized by their association with one or more autonomic features in the trigeminal nerve distribution and termed Trigeminal Autonomic Cephalalgias (TACs). Although CH is a relatively rare condition, judged by the distress it generally causes to the affected individual, early diagnosis and institution of appropriate therapy seem mandatory. Correct diagnosis of CHs needs avoidance of pitfalls. Such pitfalls generally include differentiation from migraine, differentiation from other side locked headache disorders, from other trigeminal autonomic cephalalgias (TACs), and lastly, recognition of rare presentations of cluster-like manifestations with hemiplegic aura and simulating trigeminal and glossopharyngeal neuralgias. Differentiation between primary and symptomatic CHs related to sellar pathologies and systemic medical conditions is of equal importance. In the present review such issues are discussed with the assistance of seven case vignettes.

Cáceres, Eder; Olivella, Juan Camilo; Di Napoli, Mario; Raihane, Ahmed S.; Divani, Afshin A.

doi: 10.1007/s11910-024-01382-7pmid: 39467990

Purpose of ReviewThis review aims to comprehensively examine the immune response following traumatic brain injury (TBI) and how its disruption can impact healing and recovery.Recent FindingsThe immune response is now considered a key element in the pathophysiology of TBI, with consequences far beyond the acute phase after injury. A delicate equilibrium is crucial for a healthy recovery. When this equilibrium is disrupted, chronic inflammation and immune imbalance can lead to detrimental effects on survival and disability.SummaryGlobally, traumatic brain injury (TBI) imposes a substantial burden in terms of both years of life lost and years lived with disability. Although its epidemiology exhibits dynamic trends over time and across regions, TBI disproportionally affects the younger populations, posing psychosocial and financial challenge for communities and families. Following the initial trauma, the primary injury is succeeded by an inflammatory response, primarily orchestrated by the innate immune system. The inflammasome plays a pivotal role during this stage, catalyzing both programmed cell death pathways and the up-regulation of inflammatory cytokines and transcription factors. These events trigger the activation and differentiation of microglia, thereby intensifying the inflammatory response to a systemic level and facilitating the migration of immune cells and edema. This inflammatory response, initially originated in the brain, is monitored by our autonomic nervous system. Through the vagus nerve and adrenergic and cholinergic receptors in various peripheral lymphoid organs and immune cells, bidirectional communication and regulation between the immune and nervous systems is established.

Elul, Deena; Levin, Netta

doi: 10.1007/s11910-024-01375-6pmid: 39266871

Purpose of ReviewPopulation 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 FindingsLarger 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.SummaryChanges 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.

Aquilanti, Elisa; Herrity, Elizabeth; Nayak, Lakshmi

doi: 10.1007/s11910-024-01376-5pmid: 39390309

Purpose of ReviewPrimary Central Nervous System Lymphoma (PCNSL) is an aggressive form of lymphoma that can involve the brain, spinal cord, leptomeninges and eyes. PCNSL prognosis continues to be poor, with 5-year survival rates of 30–40%. Therapeutic options are especially limited for relapsed/refractory (r/r) PCNSL. In recent years, studies shed light on the pathogenesis and oncogenic pathways driving PCNSL, leading to the development of novel therapeutics. In this review, we discuss the evidence supporting these novel agents and present ongoing clinical studies.Recent FindingsKey oncogenic drivers of PCNSL include activation of the NFkB pathway, cell cycle dysregulation, somatic hypermutation and immune evasion, leading to the investigation of targeted therapeutics and immunotherapeutics to inhibit these pathways. Such approaches include BTK inhibitors, mTOR/PI3K inhibitors, immunomodulatory agents (IMIDs), immune checkpoint inhibitors and CD19-based CAR T-cells. SummaryThe therapeutic repertoire for PCNSL is rapidly evolving, and a multi-modality approach including intensive chemotherapy regimens and novel therapies will likely be utilized in the future.

Nakhate, Vihang; Lasica, Aleksandra B.; Wen, Patrick Y.

doi: 10.1007/s11910-024-01378-3pmid: 39302605

Purpose of ReviewThe 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 FindingsIn 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.SummaryVorasidenib 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.

Meng, Huanyu; Chen, Xiaoyu; Chen, Sheng

doi: 10.1007/s11910-024-01377-4pmid: 39297918

Purpose of reviewSleep 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 findingsResearch 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 18F-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.SummarySleep 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.

Ehsani-Nia, Mohammad Ismail; Wannon, Avi; Ailani, Jessica

doi: 10.1007/s11910-024-01379-2pmid: 39320683

Purpose of ReviewMigraine 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 FindingsIt 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.SummaryAmong 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.

Reyes-Bello, Juan Sebastián; Moscote-Salazar, Luis Rafael; Janjua, Tariq

doi: 10.1007/s11910-024-01383-6pmid: 39352612

Purpose of ReviewTo evaluate the role of sedation vacations in optimizing patient outcomes and enhancing the quality of care in neurological intensive care units (ICUs). We discuss the importance of sedation management in neurocritical care, considering recent research findings and clinical guidelines.Recent FindingsRecent studies have highlighted the significance of sedation interruption protocols in improving patient outcomes in the ICU setting. Evidence suggests that daily sedation interruptions can reduce the duration of mechanical ventilation, ICU length of stay, and mortality rates. However, the implementation of these protocols requires careful consideration of patient-specific factors and a multidisciplinary approach.SummarySedation vacations play a critical role in neurocritical care by reducing mechanical ventilation duration, ICU stay length, and mortality rates. Despite the benefits, the presence of complications must be addressed to avoid adverse outcomes. Continued research is necessary to refine these strategies and improve guideline quality, ensuring safe and effective sedation management in critically ill neurological patients.

White, Nathan C.; Cowart, Christopher R.; Cios, Theodore J.

doi: 10.1007/s11910-024-01385-4pmid: 39373850

Purpose of ReviewPostoperative delirium (POD) is a common complication that has important implications for surgical patients, often leading to both short- and long-term cognitive deficits, worse outcomes, and increased healthcare costs. Given these implications, there may be a benefit in reducing the incidence of POD. Pharmacologic interventions may have the potential to reduce the risk of a patient developing POD.Recent FindingsRecently studied therapies include dexmedetomidine, propofol, haloperidol, ketamine, angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, acetaminophen, melatonin/ramelteon, corticosteroids, midazolam, physostigmine, and neostigmine. In addition, the implementation of regional anesthesia and reduction of overall anesthetic depth have been examined. Of these therapies, dexmedetomidine has been studied the most and has the most supporting evidence for prevention of POD, but current studies lack clarity on optimal dosing and timing of dexmedetomidine administration. Acetaminophen, corticosteroids, and melatonin/ramelteon are other plausible medications that have potential for reducing POD incidence, but they all require further investigation. Reduction of anesthetic depth and regional anesthetics are options for anesthetic management that show promise but still lack enough supporting evidence in recent literature to receive a strong recommendation.SummaryFuture research should focus on identifying optimal strategies for the implementation of the pharmacological options listed, including doses and timing of administration. Attention should be given to dexmedetomidine given its promise demonstrated by recent literature.