Arterial spin labeling perfusion imaging in an infant with anti-N-methyl-D-aspartate receptor encephalitis: A case...

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis characterized by complex neuropsychiatric syndromes and the presence of cerebrospinal fluid (CSF) antibodies against NMDAR. The characteristics of anti-NMDAR encephalitis in children, particularly infants, are unclear due to difficulties in neurologic assessment such as psychiatric symptoms. Additionally, subtle or non-specific findings of conventional magnetic resonance imaging (MRI) make early diagnosis even more difficult. Herein, we present the first case of infant anti-NMDAR encephalitis in which perfusion imaging demonstrated marked abnormalities and the absence of conventional MRI findings.

The patient was an 11-month-old boy who was admitted because of seizure and prolonged fever. He presented with involuntary movements of the mouth and tongue. Brain MRI showed no morphological abnormalities, but three-dimensional arterial spin labeling (ASL) perfusion imaging showed reduced blood flow in the left temporal and frontal regions and the right cerebellum. After that, a positive anti-NMDAR antibody test result was received. Despite treatment with IVIG and methylprednisolone, the involuntary movements and autonomic dysfunction gradually became more prominent. After rituximab administration, the clinical symptoms improved slightly, and follow-up MRI revealed diffuse brain atrophy and improvement in the balance of brain perfusion.

To the best of our knowledge, this is the first case report of infantile anti-NMDAR encephalitis in which cerebral blood flow was evaluated using three-dimensional ASL perfusion imaging. Indeed, our case, which showed abnormalities only in ASL perfusion imaging, suggests that CBF assessment could aid in the early diagnosis of anti-NMDAR encephalitis in infants.