[Devic's neuromyelitis optica in children: a case report and review of the literature].
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.


METHODS
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.


CONCLUSIONS
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.
Djemal
N
N
Service de pédiatrie, d'urgence et de réanimation pédiatrique, EPS Hédi-Chaker, Sfax, Tunisie.
Ben Salah
M
M
Ben Hlima
N
N
Ben Rhomdhane
W
W
Ben Ammar
H
H
Chaabouni
M
M
Chouchane
C
C
Karray
A
A
D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D005074:Evoked Potentials, Visual / Q000502:physiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009471:Neuromyelitis Optica / Q000150:complications* / Q000188:drug therapy; D010264:Paraplegia / Q000150:complications; D011239:Prednisolone / Q000031:analogs & derivatives / Q000627:therapeutic use; D013116:Spinal Cord / Q000002:abnormalities / Q000473:pathology; D016055:Urinary Retention / Q000150:complications; D014792:Visual Acuity / Q000502:physiology
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C025429:methyl prednisolonate; D011239:Prednisolone; D001379:Azathioprine