CONTINUUM Lifelong Learning in Neurology最新文献

Abstract: As newer, innovative neurology drugs enter the US health care system, neurologists should consider the cost of these treatments in addition to their efficacy, safety, and tolerability. To do so thoughtfully requires an understanding of how prescription drugs are priced in the United States. The process of drug pricing is linked to the distribution supply chain and the many stakeholders involved. Stakeholders include pharmaceutical manufacturers; wholesalers; pharmacies; pharmacy benefit managers; payers, including health insurers; hospital systems; neurologists and other clinicians; and patients. Drug pricing has taken center stage as the Inflation Reduction Act of 2022 has set maximum out-of-pocket expenses for Medicare beneficiaries for the first time in the program's history and limits drug price increases for a select group of Medicare Part D drugs. This article describes the US drug distribution supply chain and its stakeholders and introduces key drug pricing terms; a brief description of how rebates are generally estimated will also be discussed. Finally, as newer neurology outpatient drugs enter the market, the "value" of drugs will be described through cost-effectiveness terminology and their utility for the clinical neurologist.

Objective: This article provides an overview of the neurologic manifestations of sarcoidosis and select rheumatologic disorders. An approach to the assessment and differential diagnosis of characteristic clinical presentations, including meningitis and vasculitis, is also reviewed. A review of treatment options is included as well as discussion of distinct areas of overlap, including rheumatologic disease in the setting of neuromyelitis spectrum disorder and demyelinating disease in the setting of tumor necrosis factor-α inhibitors.

Latest developments: An increased understanding of the immune mechanisms involved in sarcoidosis and rheumatologic diseases has resulted in a greater diversity of therapeutic options for their treatment. Evidence directing the treatment of the central nervous system (CNS) manifestations of these same diseases is lacking, with a paucity of controlled trials.

Essential points: It is important to have a basic knowledge of the common CNS manifestations of rheumatologic diseases and sarcoidosis so that they can be recognized when encountered. In the context of many systemic inflammatory diseases, including systemic lupus erythematosus, IgG4-related disease, and sarcoidosis, CNS disease may be a presenting feature or occur without systemic manifestations of the disease, making familiarity with these diseases even more important.

Objective: The field of autoimmune neurology is rapidly evolving. This article reviews the epidemiology and pathophysiology as well as current approaches to clinical and paraclinical assessment, testing paradigms, and general principles of treatment.

Latest developments: Improved recognition of autoimmune diagnoses among patients who have phenotypically diverse, subacute onset neurologic presentations is facilitated by disease-specific antibody biomarker discovery. These antibodies have varying associations with paraneoplastic causation (from no association to greater than 70% positive predictive value), immunotherapy responses, and outcomes. To simplify assessment in an increasingly complex discipline, neurologic phenotype-specific serum and CSF antibody evaluations are recommended. Clinical trials have led to the approval of monoclonal therapies for neuromyelitis optica spectrum disorder (NMOSD) and are underway for N-methyl-d-aspartate (NMDA) receptor and leucine-rich glioma inactivated protein 1 (LGI1) encephalitides.

Essential points: Autoimmune neurology is now a mainstream subspecialty, consisting of disorders with diverse presentations detectable using antibody testing of serum and CSF. Early and sustained immunotherapy (eg, corticosteroids, intravenous immunoglobulin [IVIg], plasma exchange) is recommended and may be supplemented by immune suppressants (eg, rituximab or cyclophosphamide) to sustain responses and optimize outcomes.

Objective: Management of stroke due to large vessel occlusion (LVO) has undergone unprecedented change in the past decade. Effective treatment with thrombectomy has galvanized the field and led to advancements in all aspects of care. This article provides a comprehensive examination of neurologic intensive care unit (ICU) management of patients with stroke due to LVO. The role of the neurocritical care team in stroke systems of care and the importance of prompt diagnosis, initiation of treatment, and continued monitoring of patients with stroke due to LVO is highlighted.

Latest developments: The management of complications commonly associated with stroke due to LVO, including malignant cerebral edema and respiratory failure, are addressed, stressing the importance of early identification and aggressive treatment in mitigating negative effects on patients' prognoses. In the realm of medical management, this article discusses various medical therapies, including antithrombotic therapy, blood pressure management, and glucose control, outlining evidence-based strategies for optimizing patient outcomes. It further emphasizes the importance of a multidisciplinary approach to provide a comprehensive care model. Lastly, the critical aspect of family communication and prognostication in the neurologic ICU is addressed.

Essential points: This article emphasizes the multidimensional aspects of neurocritical care in treating patients with stroke due to LVO.

Objective: This article reviews the various conditions that can present with acute and severe central nervous system demyelination, the broad differential diagnosis of these conditions, the most appropriate diagnostic workup, and the acute treatment regimens to be administered to help achieve the best possible patient outcomes.

Latest developments: The discovery of anti-aquaporin 4 (AQP4) antibodies and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in the past two decades has revolutionized our understanding of acute demyelinating disorders, their evaluation, and their management.

Essential points: Demyelinating disorders comprise a large category of neurologic disorders seen by practicing neurologists. In the majority of cases, patients with these conditions do not require care in an intensive care unit. However, certain disorders may cause severe demyelination that necessitates intensive care unit admission because of numerous simultaneous multifocal lesions, tumefactive lesions, or lesions in certain brain locations that lead to acute severe neurologic dysfunction. Intensive care may be necessary for the management and prevention of complications for patients who have severely altered mental status, rapidly progressive neurologic worsening, elevated intracranial pressure, severe cerebral edema, status epilepticus, or respiratory failure.