Pediatric NMOSD: A Review and Position Statement on Approach to Work-Up and Diagnosis

Abstract

Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory demyelinating disease of the central nervous system (CNS) primarily affecting the optic nerves and spinal cord, but also involving other regions of the CNS including the area postrema, periaqueductal gray matter, and hypothalamus. Knowledge related to pediatric manifestations of NMOSD has grown in recent years, particularly in light of newer information regarding the importance of not only antibodies to aquaporin 4 (AQP4-IgG) but also myelin oligodendrocyte glycoprotein (MOG-IgG) in children manifesting clinically with this syndrome. In this review, we describe the current state of the knowledge related to clinical manifestations, diagnosis, and chronic therapies for children with NMOSD, with emphasis on literature that has been published in the last 5 years. Following the review, we propose recommendations for the assessment/follow up clinical care, and treatment of this population.

Pediatric demyelinating disease and anti‐MOG antibody

“…In the absence of a standard protocol for rituximab infusions, dosing in pediatric patients is variable among centers. 103…”

“…96 Recommended dosing of MMF is 500-750 mg/m 2 /dose, orally twice a day, with a maximum of 2000 mg total/day. 103,108 Considering that MMF may have a slow onset of efficacy in preventing clinical attacks, it is recommended to comedicate with oral corticosteroids for up to 6 months. 108…”

“…However, the need of chronic combination with oral steroids to sustain its modest efficacy has substantially reduced its use. 103…”

A window into the future? MRI for evaluation of neuromyelitis optica spectrum disorder throughout the disease course

“…Children with AQP4+ NMOSD generally have similar imaging features to adults with this disease, but there are unique challenges in distinguishing NMOSD from possible mimics. 92 Children with NMOSD are reported to more commonly have brain lesions and these may have a poorly demarcated, “fluffy” appearance. 32 The presence of thalamic and internal capsule lesions, which are seen in ADEM but are infrequent in NMOSD, may help to distinguish NMOSD from this important differential diagnosis in childhood.…”

“… 93 LETM is a common feature on spinal cord MRI, but may more often be caused by other neuroinflammatory conditions including MOGAD and monophasic transverse myelitis. 92 …”