Epstein - Barr virus Infection in a Patient with Neuromyelitis Optica Spectrum Disorder and Sjögren’s Syndrome: A Case Report and Review of Literature

Abstract

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.

Clinical significance of anti-SSA/Ro antibody in Neuromyelitis optica spectrum disorders

“…Based on what emerged from some observational studies, NMOSD can coexist with SS, SLE, and ATD (Levinson et al, 2018), and the 2015 IPND criteria (Wingerchuk et al, 2015) emphasized that the autoimmune comorbidity may be useful in the diagnosis of NMOSD. Our results suggest that the concomitant presence of the anti-SSA/Ro antibody in NMOSD patients might improve the accuracy of the (Manuel and Liang, 2021).…”

Clinical characteristics of Guillain–Barré syndrome in patients with primary Sjögren’s syndrome

“…In 11 patients with both primary SS and GBS reported in the literature 9 – 17 (Table 1 ), five patients were classified to have axonal injury and four patients showed demyelinating changes. In our study, the frequency of axonal damage was also slightly higher than that of demyelinating changes on electromyography.…”

“…One study identified 44 patients with primary SS from 184 patients who had polyneuropathy with limb weakness, 3 patients were diagnosed as GBS but subtype of GBS was not mentioned in the research 8 . A total of 11 patients suffered from both primary SS and GBS were found in literature as case reports (Table 1 ) 9 – 17 . The anti-SSA antibody was positive in seven of the eleven patients.…”

The causal relationship between neuromyelitis optica spectrum disorder and other autoimmune diseases

“…Saeko et al (27) showed that the CSF anti-EA-IgG titers were also higher in patients with NMOSD than in those with multiple sclerosis. Justin et al (28) reported a case of NMOSD coexisting with SS whose CSF was positive for EBV. In our study, pathways related to virus infection, such as EBV infection, were also enriched.…”