Neuromyelitis Optica and Systemic Lupus Erythematosus Association – the Chicken or the Egg Dilemma: a Case Repor

Abstract

We present a case report of a 32-year-old woman diagnosed with opticomyelitis of Devic (OMD) and systemic lupus erythematosus (SLE). The onset of neurological symptoms was with optic neuritis. Five months later the neurological deficit progressed within a few days to lower paraplegia and upper paraparesis, retention of urine and faeces, impaired somatic and deep sensation below the level of Th1 dermatome. The results from laboratory investigations confirmed anaemic syndrome, increased urea and creatinine, hypoproteinemia and severe proteinuria. The results from CSF investigations demonstrated hyperproteinorachia with extremely high Ig fractions. Serum and CSF oligoclonal bands and positive serum Aquaporin IgG 32 times higher than the upper referent limit were found. The association with SLE was confirmed by the increased levels of total ANA and anti-ds-DNA ANA. MRT visualized the spinal cord as non-homogenously hypointense on T1 and extremely hyperintense on FLAIR sequences through its whole length up to the bulbar-pontine region. The MRT findings and the serum Aquaporin IgG confirmed the diagnosis OMD. The patient was treated with intravenous immunomodulating agents. We consider the presented case of special interest because of the comorbidity of an aggressive autoimmune systemic and an organ-specific disease of the central nervous system. 

Keywords

• aquaporin IgG
• opticomyelitis of Devic
• optic neur