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Review
. 2016 May 5;129(9):1122-7.
doi: 10.4103/0366-6999.180514.

Autoimmune Encephalitis: An Expanding Frontier of Neuroimmunology

Hong-Zhi Guan, Hai-Tao Ren, Li-Ying Cui  1
Affiliations
Review

Autoimmune Encephalitis: An Expanding Frontier of Neuroimmunology

Hong-Zhi Guan et al. Chin Med J (Engl). .
No abstract available

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Figures

Figure 1
Figure 1
The laboratory experience of PUMCH encephalitis and paraneoplastic neurological syndrome project. A total of 4106 cases with encephalitis of unidentified etiology were test for antibodies against neuronal cell-surface or synaptic protein: 531 cases (12.9%) were positive for autoantibodies, including 423 cases (10.3%) with anti-NMDAR antibodies, 68 cases (1.66%) with anti-LGI1 antibodies, thirty cases (0.73%) with anti-GABABR antibodies, seven cases (0.17%) with anti-CASPR2 antibodies and three cases (0.073%) with anti-AMPAR antibodies. PUMCH: Peking Union Medical College Hospital; Ab: Antibody; NMDAR: N-methyl-D-aspartate receptor; LGI1: Leucine-rich glioma-inactivated 1; GABABR: γ-aminobutyric acid B receptor; CASPR2: Contactin-associated protein 2; AMPAR: α-amino-3-hydroxy-5-methyl-4-isoxazole propionate receptor.
Figure 2
Figure 2
CSF cytology of a patient with anti-NMDAR encephalitis. Lymphocytic inflammation is typical CSF findings of anti-NMDAR encephalitis, and plasma cells are often identified in CSF. CSF: Cerebrospinal fluid; NMDAR: N-methyl-D-aspartate receptor (May-Grunwald-Giemsa stain, original magnification ×200).
Figure 3
Figure 3
Brain MRI of autoimmune encephalitis. (a) High signals on bilateral mesial temporal lobe in a patient with limbic encephalitis associated with anti-GABABR antibodies. (b) High signals on the bilateral mesial temporal lobe and right amygdala enlargement in a patient with limbic encephalitis associated with anti-LGI1 antibodies. (c) Abnormalities in the right mesial temporal lobe in a patient with anti-NMDAR encephalitis during her initial episode. (d) New lesions more prominent at left mesial temporal lobe and brain stem during her relapse. MRI: Magnetic resonance imaging; GABABR: γ-aminobutyric acid B receptor; LGI1: Leucine-rich glioma-inactivated 1; NMDAR: N-methyl-D-aspartate receptor.
Figure 4
Figure 4
Proposed pathway for the treatment of anti-NMDAR encephalitis. The algorithm demonstrates indications of first-line, second-line, and chronic immunotherapy. The retrial of first-line therapy is an option in patients with little or no response to the initial immunotherapy. Chronic immunosuppression: mycophenolate mofetil or azathioprine for 1 year. NMDAR: N-methyl-D-aspartate receptor; IVIg: Intravenous immunoglobulin; PE: Plasma exchange.
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References

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Supplementary concepts