Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Dec 31;6(2):45-50.
doi: 10.14581/jer.16010. eCollection 2016 Dec.

The Laboratory Diagnosis of Autoimmune Encephalitis

Sang Kun Lee  1 , Soon-Tae Lee  1
Affiliations
Review

The Laboratory Diagnosis of Autoimmune Encephalitis

Sang Kun Lee et al. J Epilepsy Res. .

Abstract

Autoimmune encephalitis is a group of encephalitis syndromes that cause altered mentality, memory decline, or seizures in association with the presence of serum and cerebrospinal fluid (CSF) autoantibodies (auto-Abs). An early diagnosis enables early treatments. The detection of auto-Abs is a confirmatory diagnosis. Tissue-based assay, cell-based immunoassay, and immunoblotting are used to detect various autoantibodies. The CSF test for the presence of antibodies is important because it is more sensitive and reflects disease activity in many autoimmune encephalitis, although antibody tests can be negative even in the presence of autoimmune encephalitis. EEG is often abnormal, but nonspecific. A unilateral or bilateral medial temporal T2 high signal is a common finding in MRI. Fludeoxyglucose-positron emission tomography is sometimes useful for diagnosis in patients with normal MRI.

Keywords: Autoantibody; Autoimmune encephalitis; EEG; Imaging; Laboratory diagnosis.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
The discovery of antibodies of autoimmune encephalitis along the time axis.
Figure 2.
Figure 2.
The tissue based assay. Mouse brain tissue sections such hippocampus and cerebellum are stained with the patient’s serum or CSF by indirect immunofluorescence technique.
Figure 3.
Figure 3.
Cell-based immunoassay. The procedures are insertion of DNA cording for the target antigens into a plasmid, transfection of this plasmid into vector cells (neuronal cells), reaction of neuronal vector cells with the patient’s serum or CSF, and detection of monospecific Ab by indirect immunofluorescence.
Figure 4.
Figure 4.
The diagnostic steps to detect known and unknown antibodies.
Figure 5.
Figure 5.
The detection of novel antibodies. The detection of unknown antibodies by tissue-based assay (A and B), interaction of antibodies with neuronal cells, and confirming the presence of unknown antibodies (C and D).
Figure 6.
Figure 6.
KASPER (Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry). Samples were collected from 72 nationwide hospitals (A). The most common synaptic autoimmune encephalitis was NMDAR Ab (68%) followed by LGI1 Ab encephalitis (22%). The common onco-neuronal paraneoplatic encephalitis series were Amphiphysin, Yo, and Ma2/Ta (B).
Figure 7.
Figure 7.
Two age peaks in autoimmune encephalitis. NMDAR encephalitis had the highest peak at the third decades and LGI1 at the 7th decades.
Figure 8.
Figure 8.
Common findings of autoimmune encephalitis. Bilateral (A) or unilateral medial temporal T2 high signals (B).
Figure 9.
Figure 9.
Typical FDG-PET findings of LGI1 encephalitis. Bilateral basal ganglia hypermetabolism (A) with medial temporal hypermetabolsim (B).
See this image and copyright information in PMC

References

    1. Lancaster E. The diagnosis and treatment of autoimmune encephalitis. J Clin Neurol. 2016;12:1–13. - PMC - PubMed
    1. Bernal F, Graus F, Pifarré A, Saiz A, Benyahia B, Ribalta T. Immunohistochemical analysis of anti-Hu-associated paraneoplastic encephalomyelitis. Acta Neuropathol. 2002;103:509–15. - PubMed
    1. Lancaster E, Dalmau J. Neuronal autoantigens-pathogenesis, associated disorders and antibody testing. Nat Rev Neurol. 2012;8:380–90. - PMC - PubMed
    1. Bien CG, Vincent A, Barnett MH, et al. Immunopathology of autoanti-body-associated encephalitides: clues for pathogenesis. Brain. 2012;135(Pt 5):1622–38. - PubMed
    1. Darnell RB, Posner JB. Paraneoplastic Syndromes. New York: Oxford; 2011.