Hyperperfusion Improvement: A Potential Therapeutic Marker in Neuromyelitis Optica Spectrum Disorder (NMOSD)

TL;DR

A case study explores how hyperperfusion on MRI could serve as a useful marker for diagnosing and monitoring NMOSD in patients who cannot use contrast agents.

Contribution

Demonstrates that hyperperfusion on ASL imaging may be a viable alternative diagnostic marker for NMOSD in contrast agent-ineligible patients.

Findings

Hyperperfusion on ASL imaging improved following treatment in a patient with NMOSD.

NMOSD diagnosis was confirmed post-discharge despite initial negative AQP4-Ab ELISA results.

ASL imaging may be useful when contrast agents are contraindicated.

Abstract

A 70-year-old Japanese woman with longstanding hearing loss and asthma developed floating sensations, left finger numbness, and postural instability one day after influenza vaccination, leading to hospital admission. Neurological examinations showed hearing loss, hyperreflexia, left-predominant ataxia, bilateral mild bathyanesthesia, and inability to tandem gait. Cerebrospinal fluid (CSF) analysis showed no pleocytosis or malignant cells, but revealed positive oligoclonal bands and elevated myelin basic protein. Despite no contrast agent use due to asthma, brain magnetic resonance imaging (MRI) revealed pontine hyperintensities on diffusion-weighted imaging (DWI) and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences, along with hyperperfusion on arterial spin labeling (ASL) imaging. Serum anti-aquaporin-4 antibodies (AQP4-Ab) were negative by ELISA. Given the temporal proximity to vaccination and elevated demyelination markers, brainstem-type acute disseminated encephalomyelitis (ADEM) was initially suspected. Symptoms nearly resolved after two cycles of methylprednisolone pulse therapy. Notably, hyperperfusion gradually improved on ASL imaging. Post-discharge, a cell-based assay confirmed the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) by detecting positive anti-AQP4-Ab. She has been relapse-free for about a year without any immunosuppressants or biologics. Although contrast-enhanced MRI remains the gold standard modality for lesion evaluation due to its high sensitivity, hyperperfusion on ASL may provide a useful alternative in patients for whom contrast agents are contraindicated, such as those with asthma or impaired renal function.

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