Brain perfusion SPECT in limbic encephalitis associated with autoantibody against the glutamate receptor epsilon 2

The aim of this study was to elucidate the single-photon emission computed tomography (SPECT) pattern at the acute stage of disease in non-herpetic limbic encephalitis (NHLE) patients associated with the N-methyl-D-aspartate-type glutamate receptor epsilon 2 (GluR ϵ2) autoantibody using Z-score imaging system (eZIS) analyses.

Brain magnetic resonance imaging (MRI) and brain perfusion SPECT using technetium-99 ethyl cysteinate dimer ((99m)Tc-ECD) were performed in eight patients with NHLE (5 men and 3 women; mean age 48.8±22 years) within 20days after clinical onset.

All patients had various clinical limbic-associated symptoms and no evidence of herpes simplex infection or systemic malignancies. Two of eight patients showed abnormally hyperintense lesions on diffusion-weighted images and significant hyperperfusion in ipsilateral cerebral cortex on eZIS analysis, whereas other patients showed normal MRI findings and significant hypoperfusion in one or both sides of the limbic and paralimbic areas.

We suggest that (99m)Tc-ECD SPECT study using eZIS analyses may be helpful to detect the neuronal dysfunction, particularly in NHLE patients without abnormal MRI findings.