[Devic's neuromyelitis optica. Presentation of 2 new cases and review of the bibliography].
Devic's neuromyelitis optica is a clinical entity characterized by severe transverse myelitis, acute unilateral or bilateral optic neuropathy, no clinical involvement beyond the spinal cord or optic nerves and a monophasic or recurrent evolution. We report two cases, both female, affected by spinal cord and visual symptoms suggesting Devic's neuromyelitis. First patient, a 30 y.o. woman, was admitted due to acute flaccid tetraparesis preceded by left optic neuropathy five months before. CSF showed normal IgG level and no synthesis of oligoclonal bands. Brain and spinal cord MRI showed left optic neuropathy, signal hyperintensities at cervical and thoracic levels on the T2 weighted sequence, and diffuse enhancement after e.v. gadolinium DPTA. Second patient presented optical neuropathy at 19 years of age, transverse myelitis at 47 years of age, and a new cervical myelopathy two years later, at 49 y.o. Brain and spinal cord MRI showed bilateral optic atrophy and multiple hyperintensities among C1/C2 and C6/C7 levels, some of them cavitated. Pleocytosis, protein count of 511 mg/dl, presence of IgGs but negative oligoclonal bands was observed on CSF. In general, Devic's optic neuromyelitis has poor functional prognoses, recurrences may follow onset affecting spinal cord and optic nerves. In our two cases, we did not identify a specific cause despite all the diagnostic work-up.
Carod Artal
F J
FJ
Servicio de Neurología, Hospital SARAH-Brasilia DF, Brasil. javier@bsb.sarah.br
Brenner
C
C
Melo
M
M
del Negro
M C
MC
Pereira
R
R
D000328:Adult; D001284:Atrophy; D001766:Blindness / Q000209:etiology; D001921:Brain / Q000473:pathology; D002555:Cerebrospinal Fluid / Q000166:cytology; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000175:diagnosis; D009471:Neuromyelitis Optica / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000473:pathology; D011379:Prognosis; D011782:Quadriplegia / Q000209:etiology; D012131:Respiratory Insufficiency / Q000209:etiology; D013116:Spinal Cord / Q000473:pathology