Current Neurology and Neuroscience Reports

PURPOSE OF REVIEW: In this article, we review recent updates to the epidemiology, diagnostic testing, genetics, pathophysiology, and management of hemiplegic migraine. RECENT FINDINGS: While three genes have been historically associated with hemiplegic migraine, recent studies suggest two additional genes may also be implicated including PPRT2 and SLC1A3. Hemiplegic migraine is a severe subset of migraine with aura with symptoms including reversible hemiparesis in addition to other aura symptoms such as visual, sensory, or speech...

PURPOSE OF REVIEW: Uncommon causes of stroke merit specific attention; when clinicians have less common etiologies of stoke in mind, the diagnosis may come more easily. This is key, as optimal management will in many cases differs significantly from "standard" care. RECENT FINDINGS: Randomized controlled trials (RCT) on the best medical therapy in the treatment of cervical artery dissection (CeAD) have demonstrated low rates of ischemia with both antiplatelet and vitamin K antagonism...

PURPOSE OF REVIEW: Histiocytic disorders, including Erdheim-Chester disease (ECD), Langerhans cell histiocytosis (LCH), and Rosai-Dorfman disease (RDD), are rare neoplasms that may present with a spectrum of neurologic involvement. Diagnostic delay is common due to heterogeneity in presentation and challenging pathology. RECENT FINDINGS: Recent advances in the treatment of these diseases targeted towards mutations in the MAP kinase pathway have led to an improved prognosis in these patients with neurologic involvement...

PURPOSE OF REVIEW: The management of isocitrate dehydrogenase (IDH)-wildtype glioblastomas is an area of unmet need. Despite multimodal therapy incorporating maximal safe resection, radiotherapy, and temozolomide, clinical outcomes remain poor. At disease progression or relapse, available systemic agents such as temozolomide, lomustine, and bevacizumab have limited efficacy. We review the recent advances in the treatment of IDH-wildtype glioblastomas. RECENT FINDINGS: A broad repertoire of systemic agents is in the early stages of development, encompassing the areas of precision medicine, immunotherapy, and repurposed medications...

PURPOSE OF REVIEW: This article reviews hypopituitarism after TBI, the importance of pituitary hormones, and related controversies, concluding with a suggested patient approach. RECENT FINDINGS: While earlier studies focused on increased pituitary deficiencies after moderate-severe TBI, recent studies have focused on deficiencies after mild TBI. There has been increasing focus on the role of growth hormone after injury; growth hormone is the most frequent reported deficiency at 1 year post-TBI, and an area with unresolved questions...

PURPOSE OF REVIEW: Pronagnosia is a rare acquired or developmental pathological condition that consists of a selective difficulty to recognize familiar people by their voices. It can be distinguished into two different categories: apperceptive phonagnosia, which denotes a purely perceptual form of voice recognition disorder; and associative phonagnosia, in which patients have no perceptual defects, but cannot evaluate if the voice of a known person is or not familiar. The neural substrate of these two forms of voice recognition is still controversial, but it could concern different components of the core temporal voice areas and of extratemporal voice processing areas...

PURPOSE OF REVIEW: To review recent structural and functional MRI studies of visual hallucinations in Parkinson's disease. RECENT FINDINGS: Previously, neuroimaging had shown inconsistent findings in patients with Parkinson's hallucinations, especially in studies examining grey matter volume. However, recent advances in structural and functional MRI techniques allow better estimates of structural connections, as well as the direction of connectivity in functional MRI...

PURPOSE OF REVIEW: Isocitrate dehydrogenase (IDH) mutant gliomas are a distinct type of primary brain tumors with unique characteristics, behavior, and disease outcomes. This article provides a review of standard of care treatment options and innovative, therapeutic approaches that are currently under investigation for these tumors. RECENT FINDINGS: Extensive pre-clinical data and a variety of clinical studies support targeting IDH mutations in glioma using different mechanisms, which include direct inhibition and immunotherapies that target metabolic and epigenomic vulnerabilities caused by these mutations...

PURPOSE OF REVIEW: Recognition of node of Ranvier as the site of injury in inflammatory neuropathies contributed to discovery of antibodies against the nodal/paranodal structures. These antibodies mediate a unique type of inflammatory neuropathies that are different from typical chronic inflammatory demyelinating polyneuropathy. This review discusses the advancements made in the field of autoimmune neuropathies secondary to antibodies to nodal and paranodal proteins. RECENT FINDINGS: Neuropathies caused by antibodies to nodal-paranodal antigens including neurofascin 186, neurofascin 155, contactin1, and contactin-associated protein1 were termed as autoimmune nodopathies (AN) in 2021...

PURPOSE OF REVIEW: Purposes were to identify evidence for post-intensive care syndrome (PICS) and post-intensive care syndrome-family (PICS-F), defined as the psychological impact on families from exposure to critical care, in the neurologically injured population and to characterize existing models for neurorecovery clinics and the evidence to support their use. RECENT FINDINGS: There has been an explosion of post-ICU clinics among the general critical care population, with their use largely justified based on the management of PICS and PICS-F, terminology which excludes brain injured patients...

PURPOSE OF REVIEW: Genetic variants in GBA1 and LRRK2 genes are the commonest genetic risk factor for Parkinson disease (PD); however, the preclinical profile of GBA1 and LRRK2 variant carriers who will develop PD is unclear. This review aims to highlight the more sensitive markers that can stratify PD risk in non-manifesting GBA1 and LRRK2 variant carriers. RECENT FINDINGS: Several case-control and a few longitudinal studies evaluated clinical, biochemical, and neuroimaging markers within cohorts of non-manifesting carriers of GBA1 and LRRK2 variants...

PURPOSE OF REVIEW: The aim of this review is to summarize the current evidence on the relationship between sleep and cognition and present available data reporting the impact that sleep alterations may have on cognitive functions. RECENT FINDINGS: Research findings support the idea that sleep is involved in cognitive processes and that altered sleep homeostasis or circadian rhythms may lead to clinical and biochemical changes associated with cognitive impairment...

PURPOSE OF REVIEW: Overwhelming evidence indicates that mitochondrial dysfunction is a central factor in Parkinson's disease (PD) pathophysiology. This paper aims to review the latest literature published, focusing on genetic defects and expression alterations affecting mitochondria-associated genes, in support of their key role in PD pathogenesis. RECENT FINDINGS: Thanks to the use of new omics approaches, a growing number of studies are discovering alterations affecting genes with mitochondrial functions in patients with PD and parkinsonisms...

PURPOSE OF REVIEW: Stroke is a leading cause of death and disability worldwide. The annual incidence of new or recurrent stroke is approximately 795,000 cases per year in the United States, of which 87% are ischemic in nature. In addition to the management of modifiable high-risk factors to reduce the risk of recurrent stroke, antithrombotic agents (antiplatelets and anticoagulants) play an important role in secondary stroke prevention. This review will discuss the published literature on the use of antiplatelets and anticoagulants in secondary prevention of acute ischemic stroke and transient ischemic attack (TIA), including their pharmacology, efficacy, and adverse effects...

PURPOSE OF REVIEW: Chronic subdural hematoma (cSDH) is a common intracranial hemorrhagic disorder with a high incidence rate among the elderly. While small, asymptomatic cSDH may resolve spontaneously, surgical intervention has been the treatment of choice for larger, symptomatic cases. Surgical evacuation of cSDH may be associated with high rates of recurrence, and even asymptomatic cSDH cases tend to progress. Over the last few years, middle meningeal artery (MMA) embolization has proven to be a safe non-invasive treatment of choice with favorable outcomes and a low recurrence rate...

PURPOSE OF REVIEW: Patients with acute neurologic injury require a specialized approach to critical care, particularly with regard to sedation and analgesia. This article reviews the most recent advances in methodology, pharmacology, and best practices of sedation and analgesia for the neurocritical care population. RECENT FINDINGS: In addition to established agents such as propofol and midazolam, dexmedetomidine and ketamine are two sedative agents that play an increasingly central role, as they have a favorable side effect profile on cerebral hemodynamics and rapid offset can facilitate repeated neurologic exams...

PURPOSE OF REVIEW: Pediatric low-grade gliomas and glioneuronal tumors (pLGG) account for approximately 30% of pediatric CNS neoplasms, encompassing a heterogeneous group of tumors of primarily glial or mixed neuronal-glial histology. This article reviews the treatment of pLGG with emphasis on an individualized approach incorporating multidisciplinary input from surgery, radiation oncology, neuroradiology, neuropathology, and pediatric oncology to carefully weigh the risks and benefits of specific interventions against tumor-related morbidity...

PURPOSE OF REVIEW: There continue to be a plethora of approaches to the rehabilitation of hemispatial inattention, from different forms of sensory stimulation (visual, auditory and somatosensory feedback), through all major modes of non-invasive brain stimulation to drug therapies. Here we summarise trials published in the years 2017-2022 and tabulate their effect sizes, with the aim of drawing on common themes that may serve to inform future rehabilitative studies. RECENT FINDINGS: Immersive virtual reality approaches to visual stimulation seem well tolerated, although they have yet to yield any clinically relevant improvements...

PURPOSE OF REVIEW: It has been clear for decades that psychological factors often contribute to mild traumatic brain injury (mTBI) outcome, but an emerging literature has begun to clarify which specific factors are important, when, for whom, and how they impact recovery. This review aims to summarize the contemporary evidence on psychological determinants of recovery from mTBI and its implications for clinical management. RECENT FINDINGS: Comorbid mental health disorders and specific illness beliefs and coping behaviors (e...

PURPOSE OF THE REVIEW: The purpose of this article is to help clinicians understand how underlying pathophysiologies and medical comorbidities associated with acute traumatic brain injury (TBI) can impact assessment of cognition during the initial stages of recovery. Clinicians can use information from this article to develop assessment plans rooted in patient-centered care. RECENT FINDINGS: The authors conducted a review of the literature related to the assessment of cognition in acute TBI, focusing on pathophysiology, medical comorbidities, and assessment approaches...