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Prospective follow-up of longitudinally extensive transverse myelitis associated with schistosomiasis: the role of NMO-IgG

Background: Longitudinally extensive transverse myelitis (LETM) is defined clinically by acute transverse myelitis (ATM) and radiologically by extensive spinal cord (SC) lesions spanning three or more vertebral segments on Magnetic Ressonance Image (MRI). Even that LETM carries a considerable diagnostic challenge, has been regarded as a spectrum of Neuromyelitis optica (NMO). The seropositivity for NMO IgG, a specific biomarker of NMO, is variable in LETM patients around the world and reach 30-35% in brazilian series. LETM also is related to Neuroschistossomiasis (NS), central nervous system presentation of infection caused by a Schistossoma sp, a trematode parasite, which prevalence is around 200 million people worldwide. Definite diagnosis of NS(DNS) is based in anatomo-pathological confirmation, what is not always possible in SC presentation of NS. Probable NS (PrNS) or Possible NS (PoNS) in patients with ATM requires the presence of positive epidemiology, serologic confirmation, stool examitation, and exclusion of other causes of myelopathy. NMO IgG status and his prognostic value in LETM diagnosed as NS has not been studied. Methods: We evaluated 14 consecutive patients admitted with LETM, fulfilled diagnostic criteria to DNS(1), PrNS (9) or PoNS (4). Exclusion criteria was: systemic disase associated with LETM. All patients were tested to NMOIgG status. Patients were evaluated prospectively for developing of ON or recurrence of LETM. Results: Seven men, 11 afro-brazilian were evaluated. Median age was 30.9 (range 17-51). Median time of follow-up was 2.5 years (range 0.5 – 6). MRI showed thoracic lesion (5), thoraco-lombar (6) and entire SC lesion (2). All patients were negative for NMO IgG. 8 patients experienced recurrent LETM and one developed optic neuritis, fulfilling Wingershuk’s criteria for NMO. Conclusion: NMO-IgG is negative in this sample of Brazillian patients with LETM and presumptive diagnosis of NS. The negative status of NMO-IgG does not exclude development of recurrent LETM or NMO in this series.

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