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  • Title: [Devic's neuromyelitis optica in children: a case report and review of the literature].
    Author: Djemal N, Ben Salah M, Ben Hlima N, Ben Rhomdhane W, Ben Ammar H, Chaabouni M, Chouchane C, Karray A.
    Journal: Arch Pediatr; 2007 Nov; 14(11):1337-40. PubMed ID: 17942291.
    Abstract:
    UNLABELLED: Devic neuromyelitis optica (NMO) or Devic's syndrome is an uncommon clinical syndrome associating unilateral or bilateral optic neuritis and transverse myelitis. Usually reported in adults, childhood cases constitute a distinctive clinical entity. CASE REPORT: We report a case of NMO occurring in a 9-year-old girl, admitted for paraplegia, sphincter troubles as acute installation bladder retention and of a sudden decline of the visual acuity. Magnetic resonance imaging (MRI) revealed abnormalities of spinal cord signal with hypo intensity in T1-weighted images and hyper intensity in T2-weighted images along the spinal cord. However, the cerebral region was normal. Visual evoked potentials were consistent with retrobulbar optic neuropathy. Our patient received corticosteroids (methyl prednisolone) during 5 days followed by oral prednisone. At week three, an immunosuppressant (azathioprine) was added. Clinical outcome was favourable with disappearance of sphincter troubles, a correction of the visual acuity and a progressive disappearance of motor troubles. CONCLUSION: Pediatric Devic's NMO is rare. It is a different clinical entity with an excellent visual and neurological prognosis. Review of the literature shows that recurrence is rare in children and seems to be without long-term sequelae with corticosteroids and immunosuppressant therapy.
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