Optic neuromyelitis syndrome in Brazilian patients

被引:112
作者
Papais-Alvarenga, RM
Miranda-Santos, CM
Puccioni-Sohler, M
de Almeida, AMV
Oliveira, S
De Oliveira, CAB
Alvarenga, H
Poser, CM
机构
[1] Univ Fed Rio de Janeiro, BR-21941 Rio De Janeiro, Brazil
[2] Hosp Lagoa, Rio De Janeiro, Brazil
[3] Univ Fed Fluminense, Rio De Janeiro, Brazil
[4] Univ Fed Rio de Janeiro, CSF Lab Spc HUCFF, Rio De Janeiro, Brazil
[5] Ctr Diagnost Avancado, Rio De Janeiro, Brazil
[6] Univ Severino Sombra, Rio De Janeiro, Brazil
[7] Harvard Univ, Sch Med, Boston, MA USA
关键词
D O I
10.1136/jnnp.73.4.429
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objectives: To report the clinical features and outcome of 24 Brazilian patients with optic neuromyelitis syndrome (ONM); discuss the underlying pathological events associated with the ONM syndrome; review the nosological situation of ONM in the group of inflammatory and demyelinating diseases of the central nervous system. Patients and Methods: Patients with ONM treated at the Hospital da Lagoa, Rio de Janeiro were studied. Demographic, clinical, magnetic resonance imaging, cerebrospinal fluid, and pathological data were analysed. Results: The study consisted of 20 women, four men of whom 10 were white and 14 Afro-Brazilians. Clinical course was recurrent in 22 cases and monophasic in two. Neurological manifestations at inclusion were: sensory impairment (66%), bilateral (41.6%) or unilateral blindness (20.8%), paraplegia or quadriplegia (37.5%). The EDSS was moderate/severe in 70.8%. The underlying pathological events were respectively pulmonary tuberculosis and upper respiratory infection in the two monophasic cases; in the 22 recurrent ONM patients: pulmonary tuberculosis (3), neurocysticercosis (1), polyarteritis nodosa (1), antinuclear antibody and rheumatoid factor (1), antiphospholipid antibody primary syndrome (1), diabetes mellitus (1), hypothyroidism (1), and amenorrhea-galactorrhea (4). Normal cerebrospinal fluid was found in 52% and an inflammatory profile in 48%. Only four recurrent ONM white patients had brain and spinal cord magnetic resonance imaging and cerebrospinal fluid findings compatible with the diagnosis of multiple sclerosis. Large lesions were seen in 62% of spinal magnetic resonance images. Six of 12 recurrent ONM Afro-Brazilian died. There were no statistical differences in the demographic data of the two ethnic groups. Afro-Brazilians were significantly more severely impaired and had a higher mortality rate than the white patients. Conclusion: These cases were classified as follows: two monophasic acute disseminated encepholo-myelitis; one recurrent disseminated encephalomyelitis; three recurrent ONM associated with Hughes syndrome, autoantibodies and polyarteritis nodosa; six recurrent ONM with endocrinopathies; and finally, four multiple sclerosis cases. The remaining cases were not associated with any other condition. It would seem clear that ONM is a syndrome rather than a single disease.
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页码:429 / 435
页数:7
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