Plovdiv Medical University, Bulgaria
Title: Opticomyelitis of Devic associated with Systemic lupus erythematosus: a case report
We present a case report of a 32-year old woman diagnosed with Opticomyelitis of Devic and Systemic lupus erythematosus (SLE). The onset of neurological symptoms was with optic neuritis that affected both eyes within 2 weeks. Five months later the patient complained from paresthesias in the lower extremities and limited dorsal flexion of the right foot. The neurological deficit progressed in the following days to lower paraplegia and upper paraparesis, retention of urine and faeces, impaired somatic sensation and proprioception below the level of upper thoracic segments. The results from laboratory investigations confirmed anaemic syndrome, increased urea and creatinine, hypokalemia, hypoproteinemia with hypoalbuminemia and increased serum gamma globulines (predominantly IgG), severe proteinuria. The results from cerebrospinal fluid (CSF) investigations demonstrated hyperproteinorachia with extremely high protein fractions, low glucose and potassium levels, mild pleocytosis with increased neutrophils. Serum and CSF oligoclonal bands and positive CSF Aquaporin G 32 times higher than the upper referent limit were found. The association with Systemic lupus erythematosus was confirmed by the increased levels of rheumatoid factor, total ANA, and anti-ds-DNA ANA. MRT of the spinal cord and cranio-spinal region visualized the spinal cord as non-homogenously hypointense on T1, hyperintense on T2 and extremely hyperintense on FLAIR sequences through its whole length up to the bulbar-pontine region, without mass effect. The MRT findings and the specific CSF IgG confirmed the diagnosis Opticomyelitis of Devic. The patient was treated with intravenous immunomodulator agents: corticosteroids, immunovenin, cyclophosphamide and there was partial improvement of the neurological symptoms.
We consider the presented case is of special interest with the comorbidity of an aggressive autoimmune systemic disease and an organospecific disease of the central nervous system. Due to the treatment with immunomodulator agents, the progress of SLE was stopped, the neurological symptoms were improved, although severe neurological deficit persists.