Resumo

VARELA, Lucía et al. Mielitis longitudinal extensa de presentación muy tardía: Una entidad del espectro de la neuromielitis óptica. Medicina (B. Aires) [online]. 2020, vol.80, n.3, pp.275-279. ISSN 0025-7680.

Optic neuromyelitis spectrum diseases are inflammatory disorders of the central nervous system characterized by severe demyelination and immunomediated axonal damage that mainly affects the optic nerves and spinal cord. They usually appear at an early age, although there are some reports in the literature of patients with late presentations. We present the case of a 78-year-old woman who consulted for severe paraparesis, sensory disorders, and urinary retention. An MRI of the cervicodorsal spine was performed, showing extensive longitudinal spinal injury. Secondary causes based on clinical observations and laboratory studies were ruled out. The dosage of anti-aquaporin 4 antibodies was positive. Acute treatment with high-dose glucocorticoids and plasmapheresis was indicated, and maintenance with rituximab, obtaining little clinical response. In patients with extensive spinal injuries, multiple differential diagnoses should be considered according to the clinical presentation, findings through imaging studies and epidemiology. Likewise, it should include the search for anti-aquaporin 4 antibodies and against the oligodendrocyte myelin glycoprotein, since the functional prognosis of these patients is usually unfavourable due to the large destructive component of the lesions. Consequently, early treatment is essential in order to limit acute damage and prevent future relapses, which is especially important in late presentations of this entity due to the low functional reserve and low remyelination capacity.

Palavras-chave : Optic neuromyelitis spectrum disorders; Transverse myelitis; Longitudinally extensive transverse myelitis.

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