Pediatric Devic's neuromyelitis optica

Purpose: Neuromyelitis optica (Devic's disease) is a rare clinical syndrome of unilateral or bilateral optic neuritis (ON) and transverse myelitis (TM) occurring within an 8-week time interval. All reports mainly involve adults and describe a poor neurologic and ophthalmic clinical course without specific attention to their few pediatric cases. The purpose of this study was to develop a clinical profile in the pediatric population. Methods: A retrospective chart review over 15 years revealed nine cases of neuromyelitis optica. A literature search was undertaken and all cases of pediatric neuromyelitis optica were tabulated and analyzed separately. Results: The average age of onset was 7 years with a preceding Viral prodrome in ail patients. The optic neuritis was bilateral in eight of nine patients (89%). Visual loss ranged from 20/30 to light perception. Moderate anterior disc edema was seen in all 17 affected eyes. The clinical course was characterized by a rapid visual and neurologic recovery. The average follow up was 5.3 years, with five patients followed for 6 years or longer. None of the nine patients had any visual or neurologic recurrence or other significant subsequent illness. Visual system follow up showed 20/20 in each eye of all patients with mild optic atrophy, identified in 47% of eyes. Conclusions: Devic's neuromyelitis optica occurs in the pediatric population as a distinct clinical entity with an excellent prognosis for visual and systemic recovery and no future recurrence or long-term sequelae.