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Autoimmune Encephalitis (AIE) in Adults

This webpage is a tool to help physicians approach & manage patients suspected to have AIE based on the Canadian Consensus Guidelines for the Diagnosis and Treatment of AIE in Adults

Autoimmune Encephalitis

Autoimmune encephalitis (AIE) is an inflammatory disorder of the brain which commonly presents with subacute cognitive changes, seizures and various other neuropsychiatric manifestations

Epidemiology

AIE is not uncommon with a prevalence estimated to be comparable to infectious causes of encephalitis combined and an incidence of 0.8 -1.2 per 100,000 person-years (1-2)

Early Treatment

Early initiation of immunotherapy is associated with better outcomes in patients with AIE

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Diagnostic Evaluation in Suspected AIE

Visit this page to review the recommended investigations for patients suspected to have AIE including a checklist that can be used as a guide to help with point-of-care decision-making.

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Neural Antibody Testing

In this section, you will find information about: 

- Paired serum-CSF neural antibody testing.

- Common pitfalls in neural antibody testing interpretation.

- The importance of anti-GAD-65 antibodies titre and neurological autoimmunity.

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Treatment

In this section, you will find:

- The guidelines based recommended treatment algorithm.

- Steroids treatment plan and tapering. 

- Treatment in patients meeting only the criteria  for “Possible” AIE after completing initial investigations.

References

(1) Dubey D, Pittock SJ, Kelly CR, et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177. doi:10.1002/ana.25131

(2) Liem B, Anderson NE, Wright SL, et al. Encephalitis in adults in the Auckland and Northland regions of New Zealand, 2009 to 2018. J Clin Neurosci. 2023;107:172-177. doi:10.1016/j.jocn.2022.10.024