Current Opinion in Neurology最新文献

Purpose of review: To discuss recent advances in imaging of the structural organization and functional connectivity of central vestibular disorders with various MRI techniques. Vestibular paroxysmia, in particular the characteristics of neurovascular cross-compression of the eighth nerve, serves as an example of the peripheral vestibular disorders.

Main findings: The bilateral vestibular system is intricately connected with other sensory systems and is hierarchically organized within sensory, motor, cognitive, emotional, and memory networks. This has been demonstrated by fMRI using galvanic vestibular stimulation and fear-conditioning paradigms. Another study using transcranial electrical stimulation of the prefrontal cortex alongside galvanic vestibular stimulation showed that vestibular sensations and BOLD signals in the vestibular cortex were reduced, indicating a top-down control of vestibular input. Acute vascular cerebellar lesions around the midline and brainstem lesions affecting the vestibular nuclei can clinically mimic acute unilateral vestibulopathy; an MRI becomes clinically relevant after a few days. In unilateral thalamic infarcts, ipsilateral or contralateral tilts of perceived verticality can be differentiated by functional connectivity MRI. Rare cases of cortical rotational vertigo are due to a disconnection of interhemispheric pathways via the corpus callosum. About 30% of patients with vestibular migraine have a mild bilateral endolymphatic hydrops of the inner ear, especially in the vestibulum rather than the cochlea. Classical vestibular paroxysmia is caused by neurovascular cross-compression, which is most reliably detected by vestibular nerve angulation in DTI-MRI.

Summary: The general message of this selected review is that peripheral and central vestibular disorders do not present solely with purely vestibular signs and symptoms; they involve various levels of subcortical and cortical structures organized into neural networks.

Purpose of review: This review provides an update on recent advances in molecular and imaging biomarker discovery for the diagnosis and prognosis of vestibular schwannoma (VS), with the goal of accelerating their validation and clinical adoption.

Recent findings: A panel of nine circulating plasma biomarkers - TNF-R2/MIF/CD30/MCP-3/IL-2R/BLC/TWEAK/eotaxin/S100B - shows strong discriminatory power between patients with VS and healthy controls, with MCP-3 and S100B correlating with hearing loss and tumor size, respectively. A ~40-fold elevation of CFHR2 levels in the perilymph of patients with severe VS-induced hearing loss implicates complement activation in cochlear inflammation. Tumor-secreted TNF-α and TWEAK reach the inner ear and exhibit synergistic ototoxicity. Tissue profiling identified two distinct biomarker panels: one comprising ANGPTL1/IL17RC/LTBR/OLR1/TGFBR1, which associates with tumor cell proliferation and migration; and another including MMP-2/MMP-14/CD80/CD163/CD45, which accurately predicts peritumoral adhesion. Several tumor-derived miRNAs, including miR-431-5p, miR-7, miR-142-3p/5p, miR-155, and hypoxamiRs, are associated with hearing outcomes and tumor growth. MRI biomarkers from dynamic contrast-enhanced and diffusion-weighted imaging, as well as perilymph signal intensity ratio correlate with tumor growth, surgical outcomes, and auditory decline, respectively.

Summary: This review outlines emerging circulating, tissue-derived and imaging biomarker candidates in VS that may complement MRI and support more precise diagnosis, monitoring, and individualized management.

Purpose of review: Endovascular treatment (EVT) has dramatically improved outcomes of patients suffering from acute ischemic stroke due to large vessel occlusion (LVO), becoming the standard of care. However, up to one-third of ischemic strokes are caused by distal medium vessel occlusions (DMVO), which are beyond the LVO territory. Medical management, including intravenous thrombolysis, leaves more than half of DMVO patients disabled at 3 months, with mortality exceeding 10%. In face of this grim prognosis, expanding EVT to DMVO has gained considerable interest. This review summarizes the clinical, anatomical, and imaging features of DMVO stroke, discusses recent EVT trial results and their interpretation, and outlines future directions for establishing safe and effective reperfusion strategies in this population.

Recent findings: Recent randomized trials investigating EVT for DMVO stroke yielded neutral results overall. However, they provided important insights about patient subgroups likely to benefit from intervention and set key challenges to improving the management of patients with DMVO.

Summary: While current evidence does not support routine EVT for DMVO stroke, the field is evolving rapidly. Ongoing advances in device technology, patient selection, and trial design hold promise for refining treatment and improving outcomes in carefully selected patients.

Purpose of review: Three functional neurological disorders are encountered in neuro-otologic practice, persistent postural-perceptual dizziness (PPPD), which is the commonest cause of chronic vestibular and balance symptoms, mal de debarquement (MdDS), a rarer but potentially debilitating disorder, and functional gait disorder, an often overlooked but treatable condition.

Recent findings: Recent investigations of PPPD suggested that there may be subtypes or subthreshold variants that merit further investigation. Studies of pathological mechanisms continue to offer new insights into the complex processes that initiate and sustain the disorder, which will require nuanced models to bring together disparate findings. Evidence continues to accumulate in support of vestibulo-ocular reflex readaptation therapy for MdDS, with pilot studies offering refinements and possible alternatives. Functional gait disorder is one of the commonest manifestations of functional neurological disorder, often presenting with other functional neurological symptoms including PPPD. Specialized methods of physical and occupational therapy continue to mature. Optimal outcomes may require short and focused periods of intensive treatment.

Summary: Evolving theories and continuing emergence of new data are beginning to make functional vestibular and gait disorders a manageable part of neuro-otologic practice.

Purpose of review: Moyamoya vasculopathy is a progressive cerebrovascular steno-occlusive disease with variable presentation. As revascularization techniques, antiplatelet therapies, and imaging-based artificial intelligence (AI) diagnostics continue to advance, there is an emerging opportunity to refine patient stratification by integrating genetic profiling, neuroimaging phenotypes, and circulating biomarkers.

Recent findings: The RNF213 locus (particularly p.R4810K) represents the primary susceptibility allele in East Asian cohorts, with secondary contributors including ACTA2 and GUCY1A3 showing incomplete penetrance. Emerging. data reveal dysregulated lipid metabolism, impaired arginine-arginine-nitric oxide (NO) and methionine signaling, heightened oxidative stress, and ferroptotic pathways. Proteomic studies identify disrupted angiogenic and cytoskeletal programs with potential biomarker utility in cerebrospinal fluid and serum. Current diagnostic standards employ MRI/MRA and digital subtraction angiography. Observational data support antiplatelet agents, including cilostazol, in reducing stroke recurrence and mortality. Direct and combined bypass approaches demonstrate superior outcomes in adult hemorrhagic disease, whereas indirect revascularization predominates in pediatric populations. Emerging AI-integrated diagnostic algorithms incorporating imaging and multiomic data exhibit promising diagnostic accuracy.

Summary: Systematic integration of genotypic and multiomic profiling with hemodynamic assessment could enhance prognostic precision, optimize surgical timing, and guide antiplatelet selection in Moyamoya. Next step priorities include studying ethnically diverse multicenter registries and rigorous trials evaluating targeted and regenerative therapeutic strategies. Digital subtraction angiography (DSA)-guided diagnosis and individualized revascularization strategies remain the clinical standard.

Purpose of review: Although electroencephalography (EEG) is central to epilepsy diagnosis, its role in patients presenting with dizziness or balance disorders has historically been negligible. This review provides a timely synthesis of recent methodological and conceptual advances demonstrating how modern EEG analyses can probe cortical contributions to vestibular and balance function.

Recent findings: While vestibular epilepsy remains rare, EEG is increasingly being applied to investigate cortical dynamics during vestibular stimulation, postural control, and balance perturbations. Contemporary analytic techniques have revealed that alpha-band and beta-band EEG activity reflect key aspects of vestibular perception, adaptation, and postural control. Findings in patients with higher order vestibular dysfunction link symptoms to abnormal oscillatory patterns corresponding to disrupted sensory integration and maladaptive attentional engagement. Advances in mobile EEG approaches now permit reliable signal acquisition during movement and direct vestibular stimulation, allowing quantification of ecologically relevant cortical responses such as the perturbation-evoked potential.

Summary: EEG provides a powerful, accessible, and scalable tool to characterize cortical contributions to vestibular processing and balance. These developments highlight its emerging value for identifying neurophysiological biomarkers of vestibular dysfunction, improving diagnostic precision, and informing targeted rehabilitation strategies.

Purpose of review: To summarize recent advances in blood-based biomarkers for acute ischemic stroke relevant to diagnosis, etiological assessment, risk prediction, and outcome prognostication, and to outline future directions for clinical implementation.

Recent findings: Novel biomarkers enhance differentiation of ischemic from hemorrhagic stroke and large vessel occlusion detection, optimizing triage via point-of-care testing. Specific biomarkers improve etiological classification and identification of mechanisms like cardioembolic sources and atrial cardiopathy, enabling targeted secondary prevention. Circulating markers stratify risks of vascular recurrence and infections, linking inflammatory, thrombotic, and endothelial pathways. Prognostic biomarkers refine predictions of functional outcomes, mortality, and reperfusion responses.

Summary: To translate these promising findings into clinical care and to identify novel molecular targets, standardized sample collection, rigorous external validation, and multiomics/panel integration will be required. In this sense, blood-based-biomarkers have the potential to sustainably improve diagnostics, prognosis and treatment in stroke care.

Purpose of review: Visualization of endolymphatic hydrops using MRI has become a cutting-edge method not only for diagnosing Meniere's disease but also for pathophysiological elucidation of the disease. Here, we review recent advances in imaging analysis of the inner ear in Meniere's disease, which could provide additional information over previous findings.

Recent findings: In addition to identification of endolymphatic hydrops on MRI, high-intensity signals in the perilymph, which represent alterations in vascular permeability in the blood-perilymph barrier (BPB), might be a key to elucidating the pathophysiology of Meniere's disease. Moreover, high-intensity signals in the endolymphatic duct, which indicate disturbances of the lymph-capillary system, might provide new information for elucidating the pathogenesis of hearing loss and dizziness/vertigo not associated with endolymphatic hydrops.

Summary: Presence of endolymphatic hydrops is not necessarily the cause of symptoms related to Meniere's disease. Alteration of the BLB might lead to additional disturbances in ears with Meniere's disease and induce clinical symptoms. Disturbances of the lymph-capillary system in endolymphatic duct might induce audio-vestibular symptoms in ears without endolymphatic hydrops. Further development of MRI evaluation of inner ear conditions is desirable not only for accurate diagnosis and elucidation of the pathophysiology of the diseases, but also for early medical intervention to prevent their progression.

Purpose of review: Acute vertigo accounts for about 4% of emergency department visits in both the United States and Europe. Despite this frequency, the management of dizziness, vertigo, and balance disorders remains fragmented, with no established international care pathway. The acute vestibular syndrome (AVS) is particularly challenging, and timely recognition is essential to avoid potentially devastating outcomes. This review is timely, because misdiagnosis rates remain unacceptably high, especially for posterior circulation strokes presenting with dizziness.

Recent findings: The literature highlights a wide differential diagnosis for AVS, ranging from benign peripheral vestibular disorders to life-threatening central causes. Distinguishing stroke from peripheral disorder remains a key clinical dilemma, compounded by the limitations of early neuroimaging - MRI can yield false negatives within 48 h. Up to 35% of posterior circulation strokes with dizziness are initially missed, often by nonspecialists unfamiliar with targeted bedside tests.

Summary: A structured bedside approach, focusing on key clinical features and targeted examination, can improve diagnostic accuracy and reduce delays in appropriate treatment. Incorporating such strategies into standard practice could address a major gap in acute neurology care and improve patient outcomes.