NEUROMYELITIS OPTICA IN THE PEDIATRIC POPULATION

Objectives: Neuromyelitis optica (Devic's disease) is a distinct demyelinating syndrome characterized by the initial development of optic neuritis (typically bilateral) followed by a transverse myelitis several weeks or months later. It differs from multiple sclerosis by its particular CNS involvement, absence of oligoclonal bands and pleocytosis in the cerebrospinal fluid and imaging evidence of spinal cord involvement extending over multiple segments. This syndrome has only been reported once (Jeffery and Bundic, 1996) previously in children.

Methods: A retrospective chart review was performed on two cases with current diagnostic criteria for Devic's disease.

Results: Both patients were diagnosed at age 13 with a demyelinating disease. The first patient presented with recurrent nausea, vomiting and vertigo. A demyelinating lesion was found in his medulla on MRI. Subsequently, he had recurrent attacks of optic neuritis and transverse myelitis affecting multiple segments. Presently at age 20, he is blind and tetraplegic, requiring ventilatory support intermittently for relapses. The second patient presented initially with a bilateral optic neuritis. Shortly after his first steroid taper, he developed urinary retention and limb weakness. Spinal MRI revealed widespread demyelinating lesions. He recovered back to his baseline after each of the two attacks.

Conclusion: Neuromyelitis optica does occur in the pediatric population. It needs to be considered in the differential diagnosis of children with a demonstrated demyelinating cause for either visual loss or myelopathy.