Background: The association of Neuromyelitis Optica Spectrum Disorders (NMOSD) with autoimmune disorders including Sjögren's syndrome (SS), is well recognized. Epstein Barr virus (EBV) has been associated to various neurological entities. We describe a case where EBV infection likely preceded NMOSD in a patient with unrecognized SS. The clinical features, work up and management are described.
Case presentation: A 40-year woman with history of stroke and Guillain-Barre Syndrome (GBS) two years prior, presented with progressive lower extremity weakness and pain. Brain MRI revealed hyperintensities in the cerebellar and parietal lobes consistent with old infarcts, high intensity signal in the white matter and enhancing intramedullary lesion at the level of T2 and the conus medullaris. Cerebrospinal fluid (CSF) revealed no oligoclonal bands. Next day, the patient developed right ankle weakness and urinary incontinence. NMOSD was suspected and pulse steroids initiated. Patient's weakness resolved. Antinuclear antibodies (ANA), anti-SSA/SSB and Aquaporin 4 antibodies (AQP4Ab) were positive. CSF was positive for EBV. Parotid gland ultrasound revealed non-homogeneous tissue.Ganciclovir and plasmapheresis were started. The patient's sensation and motor deficits improved and one month after, she had regained motor power and sphincter control. The patient was discharged on oral prednisone and plans for rituximab infusions.On follow-up imaging, Spinal MRI showed areas of myelomalacia and complete resolution at the level of T2 and conus medularis lesions respectively. The patient had no additional flares, but did complain of chronic neuropathic pain.
Conclusion: NMOSD commonly coexist with other autoimmune diseases. The association of SS and NMOSD is well recognized. EBV infections can present with neurological manifestations however, EBV has also been linked to the development of autoimmunity. In our case, EBV was detected in CSF and antiviral therapy was initiated in addition to the treatment modalities for NMOSD which led to a full recovery in our patient.
Keywords: aquaporin-4 ig g antibodies; epstein-barr virus infection; neuromyelitis optica spectrum disorders; sjogren’s syndrome.