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Título

Paraneoplastic Neuromyelitis Optica Spectrum Disorder: a case report

RESUMO

CASE PRESENTATION
A 53-year-old female patient was admited to our hospital with back pain history, progressive spastic paraparesis, with urinary incontinence and constipation, being diagnosed with acute transverse myelitis. Her magnetic resonance imaging showed longitudinally extensive spinal cord lesions; and her capillary electrophoresis (CE) showed polyclonal gammopathy (PG). She received high-dose intravenous methylprednisolone with satisfactory results. During follow-up, she tested positive for AQP4-IgG, fulfilling criteria for Neuromyelitis optica spectrum disorder (NMOSD), and was discharged with Azathioprine (AZT) plus prednisone.

After 4 months, she was hospitalized again for cellulitis and infectious ulcers. Her complete blood count showed pancytopenia that was associated with the use of AZT, therefore, the medication was suspended.

The patient progressed with worsening cytopenias. Another CE disclosed monoclonal gammopathy (MG), and a myelogram was performed, which showed 60% of clonal plasma cells in the bone marrow. As she was diagnosed with Multiple Myeloma (MM), with IgA/Kappa monoclonal protein, isolated Kappa light chain and beta-2 microglobulin, AZT was discontinued and myeloma treatment started with Cyclophosphamide, Bortezomib and Dexamethasone (CYBORD protocol).

DISCUSSION
Studies report a link between autoimmune diseases accompanied by diagnosis of MG. A study described 6 cases of paraneoplastic NMOSD. In his results 5 patients were older than 50 years old, and 4 had positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months.

A case series reported a 58-year-old female with visual defects and acute transverse myelitis, with a concomitant finding of MG in CE. Similarly to our case, there were no lytic bone lesions,hypercalcemia and renal impairment.
Generally, patients with prolonged autoimmune diseases have PG. Therefore, CE with protein immunofixation and free light chain quantification is adequate for MG detection.

The pathogenesis involving inflammatory states and clonal transformation is still unclear. Polyclonal proliferation may progress to monoclonal proliferation of B cells and induce MG. Possibly, there is involvement of dysregulated cytokines.

FINAL COMMENTS
We suggest that MG should be ruled out for patients with prolonged autoimmune diseases. There is probably a direct mechanism between cancer cells and development of NMOSD, however, further studies are required.

Palavras Chave

Neuromyelitis Optica, Paraneoplastic Syndrome, Immunology

Área

Neuroimunologia