Current Neurology and Neuroscience Reports最新文献

Purpose of review: A subset of patients with headache disorders presents with a dyscapnic state typically clinically symptomatic chronic respiratory alkalosis (CSCRA). CSCRA is well described as an important factor in increased neuromuscular irritability and reduces tissue oxygen delivery through reduction of cerebral blood flow (vasospasm) and left shift of the oxygen-hemoglobin dissociation curve, as well as increasing metabolic tissue oxygen demand all thought to be important factors in headache disorders.

Recent findings: Novel testing paradigms that allow assessment of hypoxemia / orthodeoxia-platypnea include hypoxic echocardiography with bubble contrast to diagnose clinically significant right to left shunts (RLS). RLS (commonly through a patent foramen ovale (PFO)) is associated with migraine, particularly migraine with aura. We evaluate patients with an in-depth acid base analysis (including arterial blood gas analysis, comprehensive metabolic profile and urinalysis) and then pursue diagnostic evaluation of potential causative factors. Common causes of CSCRA include sleep disordered breathing, RLS (anatomic and capillary shunting), post-concussive states as well as post-viral or postoperative derangements of acid base status and associated with altered autonomic function. A focused diagnostic approach to discern the root cause for CSCRA may uncover treatable causes such as hypoxemia secondary to RLS or lung disease, sleep disorders and diaphragmatic dysfunction. The time-limited intervention with carbonic anhydrase to normalize acid base status and tissue carbon dioxide stores, as well as rehabilitation measures to normalize breathing patterns serve to normalize the acid base status, reduce neuromuscular irritability and reduce symptoms including headache.

Purpose of review: Systemic therapy is particularly important for patients with recurrent or incompletely gross-total resected ependymoma. This study focuses on summarizing the systemic therapeutic strategies including molecular targeted therapy, immunotherapy and conventional chemotherapy in recent years, with a view to bringing more advanced treatment options to patients with ependymoma.

Recent findings: In studies of systemic therapy for ependymoma, chemotherapy, although controversial, remains effective in patients with recurrent ependymoma. In targeted therapy, tyrosine kinase inhibitors, epigenetic agents and PARP inhibitors have shown promising antitumor activity in preclinical studies and some clinical trials. In immunotherapy, strategies such as immunological checkpoint inhibitors and CAR T cells therapy have shown potential therapeutic value in specific patient populations. Chemotherapy remains an important option for patients with recurrent ependymoma, and recent studies have revealed the great potential of targeted therapy and immunotherapy, suggesting that further in-depth studies on the optimization and combined application of targeted drugs and immunotherapy strategies, etc., can be conducted in the future.

Purpose of review: The frontal cortex plays a key role in many functions considered to be relevant for consciousness. Furthermore, altered consciousness appears to exist in those with disorders that disrupt or damage frontal areas and/or networks. This article reviews recent research discussing the impact of frontal disorders on consciousness.

Recent findings: Many theories of consciousness have been developed over the last century to help explain the neural correlates of consciousness. Some theories argue that the frontal cortex is necessary for consciousness, while others argue that posterior cortical regions are critical for consciousness. The Memory Theory of Consciousness argues that consciousness requires modality-specific localization throughout the brain. We argue that frontal disorders do not abolish consciousness but they may disrupt conscious abilities such as sustained attention, working memory, problem-solving, complex thought, executive function, response inhibition, decision-making, and goal-oriented behavior. Understanding the role of the frontal cortex in consciousness has significant scientific, clinical, and societal implications.

Purpose of review: This review provides a comprehensive overview of the multidisciplinary management strategies available for intracranial meningiomas, with a focus on novel therapies.

Recent findings: With the 2021 WHO classification of tumors of the Central Nervous System (CNS) came the introduction of molecular alterations impacting meningioma grading. As with other central nervous system tumors, the medical management of meningiomas is moving in the direction of targeted therapies. However, meningiomas display significant heterogeneity in their natural history and management options for advanced or recurrent tumor types are lacking. Meningioma management has advanced with refined surgical techniques, radiotherapy strategies, and emerging systemic therapies. Maximal safe resection remains the mainstay of treatment; however, several clinical challenges and unanswered questions persist. Ongoing investigations into targeted therapies and immunotherapies offer promising avenues, particularly for high-grade and recurrent cases.

Purpose of the review: This narrative review synthesizes ten key evidence-based principles for optimizing ventilatory management in patients with acute brain injury, including traumatic brain injury, stroke, and hypoxic-ischemic encephalopathy.

Recent findings: We emphasize the need to individualize ventilator settings to preserve intracranial pressure (ICP) and cerebral perfusion pressure (CPP), while maintaining lung-protective strategies. Key recommendations include prioritizing physiological targets over ventilator modes, judicious use of positive end-expiratory pressure (PEEP) with concurrent cerebral monitoring, limiting plateau pressures, and maintaining tidal volumes within protective ranges. Minimizing driving pressure (ΔP) and mechanical power (MP) is emphasized to reduce the risk of ventilator-induced lung injury (VILI). The review underscores the importance of precise control of arterial carbon dioxide (PaCO₂) to regulate cerebral blood flow, avoidance of both hypoxemia and hyperoxia, and the integration of multimodal neuromonitoring to inform ventilatory decisions. Additional considerations include the potential benefits of early tracheostomy in patients requiring prolonged ventilation, as well as the influence of sedation depth, fluid management, and autoregulation monitoring on outcomes. By aligning respiratory support with cerebral pathophysiology, clinicians can mitigate secondary brain injury and enhance recovery in this vulnerable population.

Purpose of review: This review aimed at investigating sleep abnormalities as risk factors for Alzheimer's disease (AD), with a focus on their potential utility in early disease detection and risk modification.

Recent findings: Impaired sleep quality, circadian misalignment, and disruptions in sleep architecture are significantly associated with an elevated risk of AD. Moreover, excessive or insufficient sleep, reductions in slow-wave and REM sleep, and fragmented rest-activity rhythms have been linked to early alterations in amyloid-β and tau biomarkers, even in cognitively unimpaired individuals. Various sleep disorders have also been identified as independent contributors to AD risk, particularly among genetically susceptible populations. Sleep and circadian disturbances, as well as changes in sleep architecture, represent easily detectable and modifiable risk factors for Alzheimer's disease. Integrating sleep and sleep-based metrics into preventive strategies may enhance early identification and offer novel avenues for intervention, modulating the risk of Alzheimer's disease.

Purpose of the review: Preclinical and clinical evidence support the notion that in Developmental and Epileptic Encephalopathies (DEEs) both neurodevelopmental and progressive alterations coexist. Several studies have contributed to the identification of pathophysiological mechanisms underlying DEEs, allowing novel interpretative meanings regarding the mechanisms involved in the progression of DEE symptomatology. The purpose of this review is to critically discuss emerging evidence linking DEEs, genetics, inflammation, and neurodegeneration.

Recent findings: In the last two decades, genetic findings have highlighted the role of cellular processes involved in both early neurodevelopment and neurodegeneration. In this scenario, evidence linking both autophagic and synaptic dysfunction to DEE provides the needed bridge between such pivotal processes for neuronal homeostasis and neurodegeneration. Furthermore, genetic defects affecting ion currents and cellular osmolarity, cytoskeletal structure and integrity of myelinated axons, neuronal morphology, and purine metabolism have been found to underlie DEE with progressive course. Recent evidence also demonstrates that neuroinflammation during gestation and in the immature brain can induce immune priming and increase the risk of developing DEEs. The phenotypic evolution of many DEEs beyond early childhood is still poorly understood and hardly preventable. Recent studies showed that DEEs, at least some of them, may be the consequence of early inflammatory and neurodegenerative dysfunctions with a variable severity determined by specific genetic and epigenetic factors. These findings open new avenues for uncovering the underpinnings of DEEs progression and symptomatology.

Purpose of review: This article reviews alien hand, a neurological disorder that causes involuntary limb movements that appear to be purposeful. This review outlines the history that identified the three widely accepted forms of alien hand and their distinct clinical and neuroimaging findings. This material summarizes behavioral disturbances that can occur with alien hand but are seldom addressed in a single review article, including propensity for self-injury, pathological sexual behavior, alien hand activities during sleep, and communication disturbances of alien Quality. The article then presents other paroxysmal, involuntary disturbances that are not usually considered to be alien hand, despite their appearing to be purposeful. Finally, this article reviews all PubMed-listed articles from 2020 to mid-2025 that addressed either alien hand or limb, or anarchic hand, for trends in understanding and treating this illness.

Recent findings: Meritorious advances in recent years included proposed checklists for the component behaviors for diagnosing alien hand, structured interviews for querying the patients' experiences, and demonstrating white matter cerebral damage that extends far beyond the lesion boundaries that are identifiable on conventional structural brain MRI. This review summarizes the diversity of the presentations of alien hand. Many behaviors that are encompassed by alien hand are not so far explained by clinical or experimental brain MRI findings.

Purpose of review: To outline a practical and comprehensive approach to evaluating transient neurologic dysfunction (TND) in adults.

Recent findings: TNDs are a common reason for neurologic consultation. Diagnosis relies largely on history, as neurologic examination is usually normal in the post-ictal stage. The differential of TNDs is extensive, and testing should be targeted to the more likely etiologies and ones that may portend permanent loss of neurologic function. In addition to the more common causes - transient ischemic attack (TIA), seizures, migraine auras, drug-induced adverse events, hypoglycemia, blood pressure fluctuations, hyperventilation, panic attacks, and paroxysmal vestibular disorders, there are some distinctive TND presentations and special circumstances that may point to the less common etiologies. The article outlines the key features of the common presentations and presents a comprehensive differential diagnosis that includes many rare causes of TNDs in adults and adolescents. The proposed approach relies on carefully elucidating the nature, timeline, and circumstances of the symptoms, gathering examination clues, and seeking to determine whether the event is likely due to neuro-vascular, non-vascular neurologic (paroxysmal or chronic), non-neurologic, or rare neurologic etiologies. Specific diagnoses are listed for each of these categories.