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Review
. 2012 Nov;27(11):1460-9.
doi: 10.1177/0883073812448838. Epub 2012 Aug 29.

Autoimmune Encephalitis in Children

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Free PMC article
Review

Autoimmune Encephalitis in Children

Thaís Armangue et al. J Child Neurol. .
Free PMC article

Abstract

The causes of encephalitis are numerous, and extensive investigations for infectious agents and other etiologies are often negative. The discovery that many of these encephalitis are immune mediated has changed the approach to the diagnosis and treatment of these disorders. Moreover, the broad spectrum of symptoms including, psychosis, catatonia, alterations of behavior and memory, seizures, abnormal movements, and autonomic dysregulation usually requires a multidisciplinary treatment approach. This review focuses in several forms of encephalitis that occur in children, and for which an autoimmune etiology has been demonstrated (eg, anti-N-methyl-d-aspartate receptor encephalitis) or is strongly suspected (eg, Rasmussen encephalitis, limbic encephalitis, opsoclonus-myoclonus). The authors also review several disorders that may be immune mediated, such as the rapid onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) syndrome and some encephalopathies with fever and status epilepticus. Recognition of novel immune-mediated encephalitis is important because some of these disorders are highly responsive to immunotherapy.

Conflict of interest statement

Declaration of Conflicting Interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1
Figure 1
Demonstration of neuronal antibodies with immunohistochemical techniques. Immunohistochemistry of rat brain using cerebrospinal fluid from patients with antibodies to N-methyl-D-aspartate (anti-NMDA) receptor (A), Hu (D), and a normal individual (G). The arrows point to areas shown at higher magnification in B, E, and H. Note that the pattern of reactivity of NMDA receptor antibodies (which antigen, NMDA receptor, is expressed on the cell surface and synaptic regions) is different from the pattern of reactivity of Hu antibodies (which target, HuD, is intracellular). In B, E, and H the asterisk indicates the dentate gyrus. C, F, and I show the corresponding cerebrospinal fluids reacting with live nonpermeabilized cultures of rat hippocampal neurons. Only the NMDA receptor antibodies react with the cell surface of live neurons (green staining in C); the Hu antibodies do not show reactivity because the antibodies do not penetrate the cell (F); therefore, there is lack of reactivity similar to that seen with the cerebrospinal fluid control (I). In C, F, and I, the nuclei of the neurons is shown with DAPI. (Bar in G = 2000 μm, bar in H = 100 μm, bar in I = 20 μm.) Careful assessment of novel antibodies using similar techniques is important because antibodies against cell surface antigens usually associate with syndromes that are responsive to immunotherapy whereas antibodies against intracellular antigens associate with disorders that are less treatment-responsive.

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