Current Neurology and Neuroscience Reports最新文献

Purpose of review: Rehabilitation is the mainstay of recovery after stroke, but key recommendations focused on delivering 'as much therapy as possible' and stroke survivor outcome measures have remained relatively unchanged for decades. Traditional therapy approaches focus on maximum improvement of physical impairments while a stroke survivor is in hospital to ensure that community discharge can be deemed 'safe'. This narrow approach sidelines the outcomes that are meaningful to the stroke survivor in the long term and the challenges they may face within their social context. In this article, we highlight the importance of the whole-person approach and review recent research introducing novel considerations to optimise outcomes after stroke.

Recent findings: Psychosocial well-being is a major component of health but is poorly acknowledged and managed for stroke survivors. Evidence supports the use of self-management interventions, peer befriending, and culturally - responsive methods, including deep engagement with Indigenous and cultural knowledge. Cultural safety and involvement of a stroke survivor's important personal connections are also vital for achieving truly person-centred care and equity in rehabilitation outcomes. Outcomes in rehabilitation will be optimised if we shift our mindsets from a sole focus on improving physical impairments to a broader scope of delivering whole-person care.

Purpose of review: Autosomal dominant cerebellar ataxias, also known as spinocerebellar ataxias (SCAs), are genetically and clinically diverse neurodegenerative disorders characterized by progressive cerebellar dysfunction. Despite advances in sequencing technologies, a large proportion of patients with SCA still lack a definitive genetic diagnosis. The advent of advanced bioinformatic tools and emerging genomics technologies, such as long-read sequencing, offers an unparalleled opportunity to close the diagnostic gap for hereditary ataxias. This article reviews the recently identified repeat expansion SCAs and describes their molecular basis, epidemiology, and clinical features.

Recent findings: Leveraging advanced bioinformatic tools and long-read sequencing, recent studies have identified novel pathogenic short tandem repeat expansions in FGF14, ZFHX3, and THAP11, associated with SCA27B, SCA4, and SCA51, respectively. SCA27B, caused by an intronic (GAA)•(TTC) repeat expansion, has emerged as one of the most common forms of adult-onset hereditary ataxias, especially in European populations. The coding GGC repeat expansion in ZFHX3 causing SCA4 was identified more than 25 years after the disorder's initial clinical description and appears to be a rare cause of ataxia outside northern Europe. SCA51, caused by a coding CAG repeat expansion, is overall rare and has been described in a small number of patients. The recent identification of three novel pathogenic repeat expansions underscores the importance of this class of genomic variation in the pathogenesis of SCAs. Progress in sequencing technologies holds promise for closing the diagnostic gap in SCAs and guiding the development of therapeutic strategies for ataxia.

Purpose of review: In low-grade glioma (LGG), besides the patient's neurological status and tumor characteristics on neuroimaging, current treatment guidelines mainly rely on the glioma's genetics at diagnosis to define therapeutic strategy, usually starting with surgical resection. However, this snapshot in time does not take into account the antecedent period of tumor progression and its interactions with the brain before presentation. This article reviews new concepts that pertain to reconstruct the history of previous interplay between the LGG's course and adaptive changes in the connectome within which the glioma is embedded over the years preceding the diagnosis.

Recent findings: Microscale and macroscale parameters helpful for extrapolating backward in time are considered, both for the glioma (kinetics, migration vs. proliferation profile, metabolism with possible intratumoral heterogeneity, relationships with surrounding cerebral pathways) and for patterns of reconfiguration within and across neural networks in reaction to the LGG leading to considerable interindividual cerebral variability. Modelling these continuous variations at the time of LGG diagnosis is a prerequisite to predict recovery from treatment(s). It is important to go beyond the biology of the LGG at a given moment of its history, and instead construct a more comprehensive picture of the past and present dynamics of glioma-brain interactions, and their ongoing evolution, as a necessary stage to optimize a personalized management plan by thinking several steps ahead.

Purpose of review: Early brain injury (EBI) after aneurysmal subarachnoid hemorrhage (SAH) is the most influential clinical determinant of outcomes. Despite significant advances in understanding of the pathophysiology of EBI, currently no treatments to target EBI have been developed. This review summarizes recent advances in EBI research over the past five years with a focus on potential therapeutic targets.

Recent findings: Mechanism-specific translational studies are converging on several pathophysiologic pathways: improved antioxidant delivery and the Sirt1/Nrf2 pathway for reactive oxygen species; NLRP3 inflammasome and microglial polarization for inflammation; and the PI3K/Akt pathway for apoptosis. Recently identified mechanistic components, such as microcirculatory failure and ferroptosis, need particular attention. Clinical studies developing radiographic markers and mechanism-specific, biofluid markers are attempting to bridge the translational therapeutic gap. There has been an exponential growth in EBI research. Further clinical studies which address specific pathophysiology mechanisms need to be performed to identify novel therapeutic approaches.

Purpose of review: Mobilization in the Neurological Intensive Care Unit (NICU) significantly improves outcomes and functional recovery while preventing immobility-related complications. The heterogeneity of neurologic conditions necessitates tailored, interdisciplinary mobilization strategies. This article reviews recent research on enhancing the feasibility and effectiveness of mobilization interventions in NICU settings.

Recent findings: Early mobilization improves functional outcomes, reduces complications like muscle atrophy and pressure ulcers, and can shorten ICU stays. Safe implementation involves individualized protocols and a multidisciplinary team, emphasizing that early mobilization benefits critically ill neurological patients. Development of evidenced-based protocols for interdisciplinary NICU patient mobilization enhances patient outcomes and quality of life. Use of outcome measures can facilitate mobility while preventing complications from immobility. Future research in embracing emerging technologies such as mobilization equipment and virtual/augmented reality will help determine optimal timing as well as dosage of mobility to improve long-term functional outcomes in the unique NICU population.

Purpose of review: The objective of this review is to provide a comprehensive management protocol for the treatment of intracranial pressure (ICP) crises based on the latest evidence.

Recent findings: The review discusses updated information on various aspects of critical care management in patients experiencing ICP crises, including mechanical ventilation, fluid therapy, hemoglobin targets, and hypertonic saline infusion, the advantages of ICP monitoring, the critical ICP threshold, and bedside neuromonitoring. All aspects of critical care treatment, including hemodynamic and respiratory support and adjustment of ICP reduction therapy, may impact patient outcomes. ICP monitoring allows ICP values, trends, waveforms, and CPP calculation, which are helpful to guide patient care. Advanced neuromonitoring devices are available at the bedside to diagnose impaired intracranial compliance and intracranial hypertension, assess brain function, and optimize cerebral perfusion. Future research should focus on developing appropriate intervention protocols for both invasive and noninvasive neuromonitoring in managing ICP crisis patients.

Purpose of review: To describe recent research relevant to factors which predispose to giant cell arteritis (GCA) and those which trigger its manifestation, with particular emphasis on the more recent and controversial associations (COVID-19, vaccination, novel medications) which have changed the medical landscape and perhaps GCA prevalence.

Recent findings: GCA remains more prevalent in Caucasians but nevertheless affects other racial groups. Certain HLA haplotypes (i.e. DRB1*04) incurs risk of GCA. Polymyalgia rheumatica remains a strong association, and recent evidence also associates GCA with hematologic malignancy. COVID-19 infection may trigger GCA, in addition to vaccination (particularly the COVID-19 vaccine) and reactivated VZV infection, though the latter may be related to a common trigger. PD1-inhibitors may be associated with GCA. Previously establish patterns in geography and latitude are supported. A seasonal pattern of GCA in the summer/spring months is suggested but not proven. Controversy regarding GCA risk factors exists, as well as to whether the overall prevalence of GCA is rising. Given the growing aging population, the total number of cases of GCA will certainly increase, a challenge to which that our healthcare system must continue to rise to meet.

Purpose of review: Discuss the current understanding of the pathophysiology and management of refractory trigeminal neuralgia (TN). This includes a discussion on why TN can recur after microvascular decompression and a discussion on "outside of the box" options when both first- and second-line management strategies have been exhausted.

Recent findings: This review discusses second- and third-line oral medication options, botulinum toxin A, repeat microvascular decompression, repeat ablative procedures, internal neurolysis, trigeminal branch blockade, and neuromodulation using TMS or peripheral stimulation. Additional management for chronic neuropathic facial pain such as deep brain stimulation, motor cortex stimulation, and focused ultrasound thalamotomy are also discussed, though evidence in trigeminal neuralgia is limited. Treatment of recurrent TN despite multiple surgeries can be challenging, and multiple minimally invasive and more invasive management options have been reported in small studies and case reports. Further studies are needed to determine an optimal stepwise approach.

Purpose of review: To review the management of Idiopathic Intracranial Hypertension (IIH) with co-existing conditions affecting therapy: obesity, sulfa allergy, nephrolithiasis, and pregnancy.

Recent findings: The IIH-WT trial showed that bariatric surgery is currently the most effective method for obese patients with IIH to lose weight, leading to normalization of CSF pressure in many cases. Allergy to sulfonamide antibiotics does not preclude the use of acetazolamide; rather, penicillin allergy or multiple drug allergies are the strongest predictor of a hypersensitivity reaction. Carbonic anhydrase inhibitors should be avoided in individuals with a personal history of nephrolithiasis; the risk of renal stones increases with concomitant use of other medications with the potential for nephrolithiasis. Glucagon-like peptide-1 receptor antagonists (GLP-1RA) are promising non-surgical weight loss options although preliminary studies have not demonstrated considerable impact on papilledema, headache or vision. Women with IIH have high rates of pregnancy complications partly related to obesity. Recommendations for weight gain or loss during gestation are controversial. Recent studies show better outcomes in obese women who maintain or lose weight while pregnant including gestational diabetes, pre-eclampsia and emergency caesarian section. Progress continues in the search for the cause and best treatments for IIH. Larger multicenter trials of GLP-1RA are needed to determine their efficacy.