NEUROMYELITIS OPTICA IN CHILDHOOD

Objectives: To describe features of neuromyelitis optica (NMO) in children.

Methods: A modification of the diagnostic criteria for NMO was proposed by Wingerchuk et al. in 2005. Criteria include: presence of optic neuritis (ON) and acute myelitis; removal of requirement for no clinical evidence of neurologic disease outside the optic nerves and spinal cord. Major supportive criteria: MRI evidence of contiguous spinal cord lesion extending three or more vertebral segments or NMO-IgG seropositivity.

Results: Three girls met criteria for NMO with initial symptoms developing at ages 2, 9, and 12 years. All patients developed recurrent bilateral or sequential ON that led to severe unilateral vision loss in two and moderate loss in the third. Two of the children initially presented with ADEM. The third child developed ADEM within 3 months of her ON and myelitis presentation. Cognitive testing showed moderate to severe impairments in two children. Two patients had spinal cord lesions involving 3 or more segments. The patient without spinal cord involvement had a large brainstem lesion that subsequently resolved. CSF examination was negative for oligoclonal bands in two children. NMO-IgG antibody was positive in all three patients. Some, but not all attacks, responded to intravenous steroids. Patients who experienced attacks not responsive to steroids required additional therapy. One patient responded well to mitoxantrone and later azathioprine. Two others did well with rituximab.

Conclusion: Children with NMO may have clinical features of ADEM during their course. Children with ADEM followed by recurrent ON should be tested for NMO-IgG antibodies. Among therapies considered for NMO, initial experience with rituximab appears that the medication is well-tolerated.