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Free Content Autoimmune-Mediated Encephalopathy: Classification, Evaluation, and MR Imaging Patterns of Disease

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There is growing awareness of the AMEs, an increasingly common group of disorders that lead to cognitive impairment and an array of neurologic syndromes. AMEs can mimic infection, systemic inflammatory disease, demyelinating disease, toxic-metabolic disorders, and primary neurodegenerative disorders, and the group of AMEs is often overlooked as a diagnostic consideration. AMEs can be divided into paraneoplastic and nonparaneoplastic autoimmune disorders. They can be distinguished by their association with autoantibodies, often directed against antigens in the CNS, and by the pattern of abnormalities on MR imaging. In these conditions, recognizable MR imaging patterns include limbic encephalitis, cerebellar degeneration, striatal encephalitis, brain stem encephalitis, and leukoencephalopathy. Because many of these conditions are reversible, familiarity with the clinical and imaging features may allow radiologists to recognize them and appropriately include them on the differential diagnosis. This familiarity may also facilitate the clinical work-up and treatment for these patients.
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Keywords: 18F-FDG PET = fluorine 18 fluorodeoxyglucose‐positron-emission tomography; AME = autoimmune-mediated encephalopathy; AMPAR = α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; ANNA-1 = antineuronal nuclear antibody type 1; CEC = celiac disease, epilepsy, cerebral calcifications; CNS = central nervous system; CRMP-5 = collapsin response-mediator protein-5; EEG = electroencephalography; FLAIR = fluid-attenuated inversion recovery; GABA = γ-aminobutyric acid; GABABR = γ-aminobutyric acid receptor B; GAD = glutamic acid decarboxylase; HE = Hashimoto's encephalopathy; HIV = human immunodeficiency virus; HSV = herpes simplex virus; IVIG = intravenous immunoglobulin; IgA = immunoglobulin A; IgG = immunoglobulin G; LGI1 = leucine-rich glioma-inactivated 1; MRI = MR imaging; N/A = not applicable; NMDAR = N-methyl-d-aspartate receptor; SCLC = small cell lung carcinoma; SIADH = syndrome of inappropriate anti-diuretic hormone secretion; SLE = systemic lupus erythematosus; TG = thyroglobulin; TPO = thyroperoxidase; TTG = transglutaminase; VGKC = voltage-gated potassium channel; anti-TG = anti-thyroglobulin; anti-TPO = anti-thyroperoxidase; anti-TTG = anti-transglutaminase

Document Type: Research Article

Publication date: June 1, 2011

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  • Neurographics is the peer-reviewed, bimonthly educational journal of the American Society of Neuroradiology. The journal comprises articles selected from material presented at the ASNR Annual Meeting. Neurographics also publishes other high-quality submissions that are primarily educational and have a high emphasis on a pictorial approach. Neurographics offers CME credit for reading review articles and completing quiz-based self-assessment activities.

    Visit to view all available CME courses.

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