Diagnostic criteria for anti-NMDA receptor encephalitis

Probable anti-NMDA receptor encephalitis*

All three criteria must be met:

Rapid onset (<3 months) of at least four of the six following major groups of symptoms:^¶
- Abnormal (psychiatric) behavior or cognitive dysfunction
- Speech dysfunction
- Seizures
- Movement disorder, dyskinesias, or rigidity/abnormal postures
- Decreased level of consciousness
- Autonomic dysfunction or central hypoventilation
At least one of the following laboratory results:
- Abnormal EEG (focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush)
- CSF with pleocytosis or oligoclonal bands
Reasonable exclusion of other disorders

Definite anti-NMDA receptor encephalitis*

IgG anti-GluN1 antibodies^Δ in the presence of one or more of the six major groups of symptoms, after reasonable exclusion of other disorders

NMDA: N-methyl-D-aspartate; EEG: electroencephalogram; CSF: cerebrospinal fluid; IgG: immunoglobulin G.
* Patients with a history of herpes simplex virus encephalitis in the previous weeks might have relapsing immune-mediated neurologic symptoms (post-herpes simplex virus encephalitis).
¶ In the presence of a systemic teratoma, diagnosis can be made in the presence of three groups of symptoms.
Δ Antibody testing should include testing of CSF. If only serum is available, confirmatory tests should be included (eg, live neurons or tissue immunohistochemistry), in addition to cell-based assay.

Reproduced from: Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15:391. Table used with the permission of Elsevier Inc. All rights reserved.

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Diagnostic criteria for anti-NMDA receptor encephalitis