Current Opinion in Neurology

PURPOSE OF REVIEW: Cluster headache stands among the worst debilitating pain conditions. Available treatments for cluster headache have often disabling side effects, are not tolerated, or are ineffective. The management of drug-refractory chronic forms is challenging. New treatments are warranted and reported here. RECENT FINDINGS: In cluster headache acute treatment, delivery systems like Demand Valve Oxygen or nonrebreather-type masks could enhance the effectiveness of inhaled oxygen therapy...

PURPOSE OF REVIEW: Immune checkpoint inhibitors (ICPI) and chimeric antigen receptor T cells (CAR-T) represent novel therapies recently approved to treat a number of human cancers. As both approaches modulate the immune system, they can generate a number of immune-related adverse events (irAEs), including a large spectrum of novel neurological toxicities. These are of special interest given their potential severity and risk of compromising further oncologic treatment. We aim to provide a comprehensive review of the literature and discuss their optimal management...

PURPOSE OF REVIEW: Research on multiple sclerosis (MS) pathogenesis and therapy is to a large extent driven by results obtained in experimental autoimmune encephalomyelitis (EAE). This approach provided deep insights into the mechanism of brain inflammation and immune mediated tissue injury and, thus, most of our currently established therapies for MS patients have been developed with profound contributions of experimental autoimmune research. Recent data, which are summarized in this review article, however, show important differences between EAE and MS...

PURPOSE OF REVIEW: The diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) has been revised in the past 20 years and pathological and therapeutic data have been accumulated. This review provides an overview of evolution and broadening of the concept of NMOSD. RECENT FINDINGS: NMOSD encompassing brain syndrome as well as optic neuritis and acute myelitis is now classified into aquaporine-4 (AQP)-antibody-seropositive and aquaporine-4 (AQP)-antibody-seronegative diseases, detecting more patients earlier than before...

PURPOSE OF REVIEW: Migraine is a primary headache disorder and one of the most common and disabling neurological diseases worldwide. Genome-wide association studies have identified ≈40 genetic loci associated with migraine. How these and other genetic findings are used to expand our knowledge on the pathophysiological mechanism of common migraine and rare migraine variants will be discussed. RECENT FINDINGS: The genetic load, based on common polygenic variation, is higher in familial migraine cases than in nonfamilial cases, and higher for migraine with aura and hemiplegic migraine...

PURPOSE OF REVIEW: Migraine is a common, highly disabling disorder. Its treatment involves acute and preventive therapy. Many of available preventive medications are not well tolerated, which results in poor compliance and limited effectiveness. Cannabinoids have been proposed for the treatment of migraine but their efficacy and tolerability are controversial. RECENT FINDINGS: Cannabinoids modulate functions and activity of signaling pathways that have a key role in pain control...

PURPOSE OF REVIEW: The trigeminal autonomic reflex is a physiological reflex with an important protective function which also plays a role in pathophysiological conditions, such as primary headache. It is not understood whether the autonomic symptoms in trigeminal autonomic cephalalgias and migraine are the consequence of severe trigeminal discharge or indeed directly driven by central generators as part of the pathophysiology, underlying these syndromes. RECENT FINDINGS: Modulating this reflex, and particularly the parasympathetic reflex arc, has been shown to be effective in treating headache...

PURPOSE OF REVIEW: Medications for relapsing multiple sclerosis (MS) effectively reduce relapse rate, mitigate disability progression and improve MRI measures of inflammation. However, they have virtually no impact on remyelination which is the major mechanism preventing MS-associated neurodegeneration. Stimulating the generation of myelin-(re)producing cells is therefore a central focus of current MS research and a yet unmet clinical need. Here, we present and evaluate key scientific studies from the field of (therapeutic) remyelination research covering the past 1...

PURPOSE OF REVIEW: The current article appraises the recent developments in idiopathic intracranial hypertension (IIH), with particular attention to novel therapeutic avenues and advanced clinical assessment and monitoring with optical coherence tomography and telemetric intracranial pressure devices. RECENT FINDINGS: The incidence of IIH is increasing. The first consensus guidelines for IIH have been published detailing investigation and management algorithms for adult IIH...

PURPOSE OF REVIEW: Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality. RECENT FINDINGS: Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease...

PURPOSE OF REVIEW: Deep brain stimulation (DBS) is an established but growing treatment option for multiple brain disorders. Over the last decade, electrode placement and their effects were increasingly analyzed with modern-day neuroimaging methods like spatial normalization, fibertracking, or resting-state functional MRI. Similarly, specialized basal ganglia MRI sequences were introduced and imaging at high field strengths has become increasingly popular. RECENT FINDINGS: To facilitate the process of precise electrode localizations, specialized software pipelines were introduced...

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PURPOSE OF REVIEW: Cluster headache is by many regarded as a males' disorder that is often accompanied by an unhealthy lifestyle. We aimed to study the influence of sex and lifestyle factors on clinical presentation, the diagnostic process and management. RECENT FINDINGS: Overall, the clinical presentation of cluster headache in both sexes was similar; however, chronic cluster headache may occur more frequently in women than in men. Misdiagnosis was most prevalent in women and more women than men were not correctly diagnosed until seen in a highly specialized center...

PURPOSE OF REVIEW: To review the clinical findings, differential diagnosis, treatment and outcome of pseudotumoral demyelinating lesions including tumefactive demyelination and Baló's concentric sclerosis. RECENT FINDINGS: MRI findings, such as dynamic restricted diffusion changes at the edge of pseudotumoral lesions help to discriminate atypical demyelination from key differential diagnoses, and together with histopathological data, indicate that tissue hypoxia may be important aetiologically...

PURPOSE OF REVIEW: The clinical interest for auto-antibodies against myelin oligodendrocyte glycoprotein (MOG) has recently reemerged, with the use of more specific detection methods. Large national cohorts have allowed characterizing a more precise clinical spectrum delineated by the presence of human MOG-antibodies. RECENT FINDINGS: In adults with MOG-antibodies, optic neuritis is the most frequent clinical presentation, with features different from multiple sclerosis (MS), including bilateral involvement and predilection for the anterior part of the optic nerve...

PURPOSE OF REVIEW: This study, taking the example of Alzheimer's and Parkinson's diseases, presents the experimental and human data that support the hypothesis that Aβ, tau, and α-synuclein may seed and propagate the pathology and consider the potential clinical consequences. RECENT FINDINGS: Aβ aggregates transmit Aβ pathology to experimental animals. Interhuman transmission of Aβ pathology has also been observed in iatrogenic Creutzfeldt-Jakob disease, or after dural graft...

PURPOSE OF REVIEW: To describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. RECENT FINDINGS: Affected patients present with symptoms of one or more of meningitis (headache and neck ache), encephalitis (delirium, tremor, seizures, or psychiatric symptoms), and myelitis (sensory symptoms and weakness). Optic disc papillitis (blurred vision) is common...

PURPOSE OF REVIEW: The current review develops the clinical presentations of nonparaneoplastic autoimmune cerebellar ataxia (ACA) and analyzes the association with autoantibodies. RECENT FINDINGS: Emerging evidence suggests that autoimmunity is involved in a significant proportion of sporadic ataxia cases. Moreover, numerous autoantibodies have recently been described in association with sporadic cerebellar ataxia, improving diagnosis and patient categorization...

PURPOSE OF REVIEW: Multiple sclerosis (MS) and related autoimmune disorders of the central nervous system such as neuromyelitis optica spectrum disorders (NMOSD) are characterized by chronic disability resulting from autoimmune neuroinflammation, with demyelination, astrocyte damage, impaired axonal transmission and neuroaxonal loss. Novel therapeutics stopping or reversing the progression of disability are still urgently warranted. This review addresses research on optic neuritis in preclinical experimental models and their translation to clinical trials...

PURPOSE OF REVIEW: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease...