Abstract
There are several well-described and studied autoimmune diseases that affect different organ systems, and a limited number of these affect the central nervous system. Autoimmune encephalitis represents a disease with a wide spectrum of clinical manifestations and different levels of severity, from mild cognitive impairment to complex encephalopathy.Immune-mediated encephalitis refers to a diverse and rare group of conditions in children associated with nonspecific symptomatology, altered mental state, and recalcitrant seizures. Infectious etiology must be excluded. Immune-mediated encephalitis syndromes could be associated with paraneoplastic or primarily autoimmune mechanisms. The newest scientific advantages have concluded that autoimmune encephalitis may be further divided into different groups of diseases depending on the immune response; examples are antibodies to cell surface proteins, antibodies to intracellular synaptic proteins, T-cell response with antibodies to intracellular antigens, among others. Treatment consists of supportive therapy, ranging from supplemental oxygen, fluid restriction to mechanical circulatory support. Specific treatment includes immunoglobulin infusion, plasmapheresis, and pulse steroid treatment. Prognosis is poor if specific treatment is not timely instituted.The diagnosis of autoimmune encephalitis could be challenging to clinicians due to its diverse clinical features, which can mimic a variety of other pathologic processes. Screening for cancer and proper management that includes immune therapy are fundamental, although some patients will need immune suppression for weeks or months as autoimmune encephalitis may relapse; therefore, follow-up is always necessary.
Highlights
BackgroundEncephalitis is an inflammatory condition of the brain with multiple etiologies [1,2]
The newest scientific advantages have concluded that autoimmune encephalitis may be further divided into different groups of diseases depending on the immune response; examples are antibodies to cell surface proteins, antibodies to intracellular synaptic proteins, T-cell response with antibodies to intracellular antigens, among others
Autoimmune encephalitis syndromes (AESs) describe a basket of encephalitis entities associated with antibodies against the neuronal cell surface or synaptic proteins [2,3,4]
Summary
Encephalitis is an inflammatory condition of the brain with multiple etiologies [1,2]. Characteristic MRI findings in patients with paraneoplastic or autoimmune encephalitis include signal hyperintensities on fluid-attenuated inversion recovery (FLAIR) or T2-weighted images in medial temporal lobes and/or brainstem; subcortical regions and the cerebellum are sometimes affected. Classic temporal lobe lesions on magnetic resonance (MR) imaging with the expected clinical findings of limbic encephalitis plus additional features of stiff person syndrome Îł-Aminobutyric Acid Receptor (GABAr) encephalitis: less common but better overall prognosis subtypes of Îł-aminobutyric acid A-receptor or B-receptor subunits Îł-aminobutyric acid B-receptor classic features of limbic encephalitis defined by early and frequent seizures with the development of T2-FLAIR hyperintense signal changes in 1 or both temporal lobes seen with small-cell lung cancer or pulmonary neuroendocrine tumors responds well to immunosuppression and removal of the underlying tumor Îł-aminobutyric acid A-receptors good prognosis with adequate treatment not associated with cancer extensive T2-FLAIR hyperintense lesions outside of the limbic system
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