Clinical and neuroradiological findings in patients with Anti-AMPAR encephalitis: associations with outcomes.

BACKGROUND

Encephalitis associated with antibodies against the α-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor (AMPAR) is an extremely rare type of antibody-mediated encephalitis. Its clinical phenotype and neuroradiological characteristics remain incompletely described.

METHODS

We present four cases of anti-AMPAR encephalitis, and a comprehensive literature review highlighted the diverse clinical experiences, with specific attention to the clinical and radiographic characteristics of anti-AMPAR encephalitis, as well as the association between clinical, neuroradiological presentations and outcomes.

RESULTS

A total of 89 patients with anti-AMPAR encephalitis were included in this review. Clinical presentations at the onset of anti-AMPAR encephalitis were diverse, including behavioral, cognitive, motor, and sensory manifestations. Neuroradiological findings cannot be restricted to the limbic system. They may spread to unexpected sites, like basal ganglia, cerebellum, cerebral cortex regions, and even diffuse hyperintensities, or patient may have completely normal brain magnetic resonance imaging (MRI). Fisher’s exact test showed that there was non-significant difference in the clinical symptoms and outcomes among different MRI presentations.Logistic regression analysis showed no significant correlations between clinical, neuroradiological findings and unfavorable outcome.

CONCLUSION

Anti-AMPAR encephalitis mainly presents as limbic encephalitis, and most of the patients had positive brain MRI results. The range of hyperintensity on MRI may not be directly associated with patients’ clinical symptoms and outcomes. For the expanding clinical profile of encephalitis associated with antibodies against AMPAR, careful databasing of new cases will facilitate more definitive study in the future.