Arterial Spin Labeling Imaging Characteristics of Anti-leucine-rich Glioma-Inactivated 1 Encephalitis: A Qualitative and Quantitative Analysis.

BACKGROUND AND SIGNIFICANCE

Autoimmune encephalitis (AE) is a rare group of diseases that can present with stroke-like symptoms. Anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis is an AE subtype that is infrequently associated with neoplasms and highly responsive to prompt immunotherapy treatment. Therefore, accurate diagnosis of LGI1 AE is essential in timely patient management. Neuroimaging plays a critical role in evaluating stroke and stroke mimics such as AE. Arterial Spin Labeling (ASL) is an MRI perfusion modality that measures cerebral blood flow (CBF) and is increasingly used in everyday clinical practice for stroke and stroke mimic assessment as a non-contrast sequence. Our goal in this preliminary study is to demonstrate the added value of ASL in detecting LGI1 AE for prompt diagnosis and treatment.

METHODS

In this retrospective single center study, we identified six patients with seropositive LGI1 AE who underwent baseline MRI with single delay 3D pseudocontinuous ASL (pCASL), including five males and one female between ages 28 and 76 years, with mean age of 55 years. Two neuroradiologists qualitatively interpreted the ASL images by visual inspection of CBF using a two-point scale (increased, decreased) when compared to both the ipsilateral and contralateral unaffected temporal and non-temporal cortex. The primary measures on baseline ASL evaluation were a) presence of ASL signal abnormality, b) if present, signal characterization based on the two-point scale, c) territorial vascular distribution, d) localization, and e) laterality. Quantitative assessment was also performed on postprocessed pCASL cerebral blood flow (CBF) maps. The obtained CBF values were then compared between the affected temporal cortex and each of the unaffected ipsilateral parietal, contralateral temporal, and contralateral parietal cortices.

RESULTS

On consensus qualitative assessment, all six patients demonstrated ASL hyperperfusion and corresponding FLAIR hyperintensity in the hippocampus and/or amygdala in a non-territorial distribution (6/6, 100%). The ASL hyperperfusion was found in the right hippocampus or amygdala in 5/6 (83%) of cases. Four of the six patients underwent initial follow-up imaging where all four showed resolution of the initial ASL hyperperfusion. In the same study on structural imaging, all four patients were also diagnosed with mesial temporal sclerosis (MTS). Quantitative assessment was separately performed and demonstrated markedly increased CBF values in the affected temporal cortex (mean, 111.2 ml/min/100 g) compared to the unaffected ipsilateral parietal cortex (mean, 49 ml/min/100 g), contralateral temporal cortex (mean, 58.2 ml/min/100 g), and contralateral parietal cortex (mean, 52.2 ml/min/100 g).

DISCUSSION

In this preliminary study of six patients, we demonstrate an ASL hyperperfusion pattern, with a possible predilection for the right mesial temporal lobe on both qualitative and quantitative assessments in patients with seropositive LGI1. Larger scale studies are necessary to further characterize the strength of these associations.