Pediatric Relapsing Anti-NMDAR Encephalitis Crossing with Tumefactive Demyelinating Lesion

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalopathy characterized by neuropsychiatric symptoms, autonomic instability, and abnormal movements. Most children who undergo tumor removal and receive appropriate immunosuppressants have substantial neurological improvement. We report a 14-year-old girl with relapsing anti-NMDAR encephalitis who presented with paroxysmal tingling and weakness of the right lower limb. Despite long-term prophylaxis with immunotherapy (azathioprine), tumefactive demyelinating lesion and multifocal subcortical white matter lesions were identified on magnetic resonance imaging, which improved after high doses of steroid therapy. There may be overlap between the clinical manifestations of anti-NMDAR encephalitis and acquired demyelination syndromes in children.

Tumefactive Demyelinating Lesions in Children: A Rare Case of Conus Medullaris Involvement and Systematic Review of the Literature

“…Although the presence of multiple tumefactive lesions was not generally associated with a diagnosis of multiple sclerosis, the presence of non-tumefactive lesions was associated with such a diagnosis in 10 of 11 children. Consistent with previous reports, 36 ,49 evaluation of lesion location demonstrated frontoparietal involvement in most cases, whereas cerebellar, brainstem, and spinal cord involvement was uncommon.…”

“…The clinical, demographic, and radiologic features of this pediatric cohort are illustrated in (Figure 3 9 –48 ). A detailed summary of each case is shown in Online Appendix Table 1.…”