Citations

AbstractPrompt recognition of an inflammatory myelopathy is critical, as a specific diagnosis and management plan allows for optimal patient outcomes. Many treatment options are now available for autoimmune and paraneoplastic myelopathies, but specific management strategies and expected prognosis vary widely depending on the underlying etiology. An understanding of the relevant clinical details, imaging findings, and other diagnostic information that can help achieve a specific myelopathy diagnosis and treatment plan is essential for all neurologists, given the variety of contexts in which myelopathies are encountered. We provide an outline of the diagnostic evaluation and treatment of various inflammatory myelopathies seen in autoimmune and paraneoplastic diseases, including multiple sclerosis, aquaporin-4 immunoglobulin G (IgG) seropositive neuromyelitis optica spectrum disorder, sarcoidosis, myelin oligodendrocyte glycoprotein IgG associated disease, and other rare inflammatory myelopathies; we also highlight common mimickers of inflammatory myelopathies.

AbstractThe field of autoimmune neurology is evolving rapidly. The discovery of autoantibodies that target neural antigens has expanded swiftly in the last decade. Recognition of the clinical syndromes associated with autoimmune neurologic disorders, and our understanding of the pathophysiology, has progressed significantly. Radiographic, electrophysiological, and laboratory testing (particularly neural autoantibody testing) are fundamental in the diagnosis of autoimmune neurological disorders and in the exclusion of mimics. Furthermore, investigations may serve as a baseline from which objective assessment of improvement or detection of relapse can be made. These disorders can be associated with underlying neoplasms, and screening for malignancy is an essential component of the investigations. Early diagnosis and prompt initiation of immunotherapy can improve neurologic function. The use of immunotherapy, however, can be associated with diverse side effects, and careful monitoring is crucial to prevent complications. Herein the authors address the diagnostic and treatment approach of autoimmune neurologic disorders, with particular focus on antibody-mediated neurologic autoimmunity.

AbstractTo determine whether CSF leucine‐rich glioma‐inactivated 1(LGI1)‐IgG titer, index or IgG subclass has prognostic significance, we tested serum and CSF specimens collected concomitantly from 39 seropositive patients. LGI1‐IgG index was elevated (>1) in 21 patients (54%), suggesting intrathecal synthesis. Patients with worse outcome at last follow‐up (modified Rankin Scale >2) had significantly higher index (median 6.57 vs. 0.5, P = 0.048) compared to those with better outcome. Higher CSF LGI1‐IgG4 subclass‐specific titer and index correlated with worse outcome (P < 0.005 for both). These data suggest that evidence of intrathecal LGI1‐IgG synthesis may correlate with neuronal injury and warrant consideration of aggressive immunotherapy.

Every neurologist will be familiar with the patient with atypical spinal cord disease and the challenges of taking the diagnosis forward. This is predominantly because of the limited range of possible clinical and investigation findings making most individual features non-specific. The difficulty in obtaining a tissue diagnosis further contributes and patients are often treated empirically based on local prevalence and potential for reversibility. This article focuses on improving the diagnosis of adult non-traumatic, non-compressive spinal cord disorders. It is structured to start with the clinical presentation in order to be of practical use to the clinician. We aim, by combining the onset phenotype with the subsequent course, along with imaging and laboratory features, to improve the diagnostic process.

Objective To evaluate the incidence and prevalence of autoimmune encephalitis and compare it to that of infectious encephalitis. Methods We performed a population‐based comparative study of the incidence and prevalence of autoimmune and infectious encephalitis in Olmsted County, Minnesota. Autoimmune encephalitis diagnosis and subgroups were defined by 2016 diagnostic criteria, and infectious encephalitis diagnosis required a confirmed infectious pathogen. Age‐ and sex‐adjusted prevalence and incidence rates were calculated. Patients with encephalitis of uncertain etiology were excluded. Results The prevalence of autoimmune encephalitis on January 1, 2014 of 13.7/100,000 was not significantly different from that of all infectious encephalitides (11.6/100,000; p = 0.63) or the viral subcategory (8.3/100,000; p = 0.17). The incidence rates (1995–2015) of autoimmune and infectious encephalitis were 0.8/100,000 and 1.0/100,000 person‐years, respectively ( p = 0.58). The number of relapses or recurrent hospitalizations was higher for autoimmune than infectious encephalitis ( p = 0.03). The incidence of autoimmune encephalitis increased over time from 0.4/100,000 person‐years (1995–2005) to 1.2/100,000 person‐years (2006–2015; p = 0.02), attributable to increased detection of autoantibody‐positive cases. The incidence (2.8 vs 0.7/100,000 person‐years, p = 0.01) and prevalence (38.3 vs 13.7/100,000, p = 0.04) of autoimmune encephalitis was higher among African Americans than Caucasians. The prevalence of specific neural autoantibodies was as follows: myelin oligodendrocyte glycoprotein, 1.9/100,000; glutamic acid decarboxylase 65, 1.9/100,000; unclassified neural autoantibody, 1.4/100,000; leucine‐rich glioma‐inactivated protein 1, 0.7/100,000; collapsin response‐mediator protein 5, 0.7/100,000; N‐methyl‐D‐aspartate receptor, 0.6/100,000; antineuronal nuclear antibody type 2, 0.6/100,000; and glial fibrillary acidic protein α, 0.6/100,000. Interpretation This study shows that the prevalence and incidence of autoimmune encephalitis are comparable to infectious encephalitis, and its detection is increasing over time. Ann Neurol 2018;83:166–177