CONTINUUM Lifelong Learning in Neurology最新文献

Objective: This article provides a framework for the initial evaluation of patients with suspected peripheral nerve disease. The key clinical elements of peripheral nerve diseases can help the practicing neurologist differentiate among peripheral neuropathies with similar presentations.

Latest developments: The wide range of peripheral nerve diseases with similar clinical presentations can pose a diagnostic challenge. The large array of available testing modalities (including imaging and electrodiagnostic, autonomic, laboratory, biopsy, and genetic testing) further complicates clinical decision making. Recent developments (eg, discovery of new autoantibodies, genetic variations, and histopathologic techniques) across the peripheral neuropathy spectrum have resulted in an increased need to evaluate patients logically and with a tailored diagnostic approach.

Essential points: A careful approach that focuses on key clinical elements combined with an understanding of purposeful diagnostic testing can lead to a successful diagnosis of peripheral nerve diseases.

Objective: This article reviews the etiologies, presentations, and management of neuropathies related to nutritional deficiencies.

Latest developments: Peripheral neuropathy can be the predominant or only manifestation of certain nutrient deficiencies. Cognitive difficulties or involvement of other parts of the central nervous system, such as the optic nerve and spinal cord, may accompany nutritional peripheral neuropathies. In most patients, the nutritional deficiency may have a single predominant cause, but in some cases, multiple causes may coexist. Obesity, for unclear reasons, can be associated with nutrient deficiencies. The rising rates of bariatric surgery and the incidence of nutrient deficiencies following bariatric surgery make this a particularly relevant topic for neurologists.

Essential points: Neuropathies caused by nutrient deficiencies are preventable with appropriate supplementation in high-risk situations. Early recognition and prompt treatment are essential to ensure an optimal outcome and minimize neurologic morbidity.

Objective: This article reviews autoimmune axonal neuropathies, their characteristic clinical features, disease and antibody associations, appropriate ancillary testing, treatment, and prognosis.

Latest developments: In 2021, the American College of Rheumatology and the Vasculitis Foundation released new summary guidelines for the treatment of antineutrophil cytoplasmic autoantibody-associated vasculitides. In addition, novel autoantibodies have been recently identified; they are often paraneoplastic and associated with axonal neuropathies.

Essential points: Recognition of autoimmune axonal neuropathies is important because of the potential for effective treatment to either reverse deficits or slow the progression of disease. It is necessary to properly assess for associations with other systemic disorders (eg, systemic vasculitis, connective tissue disease, neoplasm) so that adequate treatment for both neurologic and non-neurologic aspects of the disease can be initiated.

Objective: This article provides an overview of hereditary neuropathies, describes the different hereditary neuropathy subtypes and the clinical approach to differentiating between them, and summarizes their clinical management.

Latest developments: Increasingly available clinical genetic testing has broadened the clinical spectrum of hereditary neuropathy subtypes and demonstrated a significant overlap of phenotypes associated with a single gene. New subtypes such as SORD -related neuropathy and CANVAS (cerebellar ataxia, neuropathy, vestibular areflexia syndrome) have emerged. The optimization of clinical management has improved gait and motor function in the adult and pediatric populations. Novel therapeutic approaches are entering clinical trials.

Essential points: Hereditary neuropathies constitute a spectrum of peripheral nerve disorders with variable degrees of motor and sensory symptoms, patterns of involvement, and clinical courses.

Objective: This article discusses the clinical manifestations and management of infectious peripheral neuropathies.

Latest developments: Several infectious etiologies of peripheral neuropathy are well-recognized and their treatments are firmly established. The COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with several central and peripheral nervous system manifestations, including peripheral neuropathies. Additionally, some COVID-19 vaccines have been associated with Guillain-Barré syndrome. These disorders are an active area of surveillance and research. Recent evidence-based guidelines have provided updated recommendations for the diagnosis and treatment of Lyme disease.

Essential points: Infectious agents of many types (primarily bacteria and viruses) can affect the peripheral nerves, resulting in various clinical syndromes such as mononeuropathy or mononeuropathy multiplex, distal symmetric polyneuropathy, radiculopathy, inflammatory demyelinating polyradiculoneuropathy, and motor neuronopathy. Knowledge of these infections and the spectrum of peripheral nervous system disorders associated with them is essential because many have curative treatments. Furthermore, understanding the neuropathic presentations of these disorders may assist in diagnosing the underlying infection.