Although treatment strategies for NMO and/or NMOSD and systemic autoimmune disease are comparable and may overlap, care must be taken when treatment involves use of biologic agents targeting tumor necrosis factor and its receptors because there have been reports of associated CNS demyelinating events. dose immunosuppressive therapy, she failed to regain vision in one eye. Conclusion Patients presenting with optic neuritis and severe visual loss should be screened for neuromyelitis optica and treated appropriately. Neuromyelitis optica has been associated with systemic autoimmune diseases, in particular Sj?grens syndrome, and current evidence indicates that they are two distinct entities. We APG-115 recommend that both diagnoses be considered in cases of optic neuritis with severe visual loss. Introduction Optic neuritis (ON), although uncommon, can be the initial presentation of Sj?grens syndrome (SS) [1]. Coexisting SS has also been reported in 2% to 30% of patients with neuromyelitis optica (NMO) [2]. NMO is an inflammatory demyelinating disorder of the central nervous system (CNS) characterized by recurrent attacks of ON and longitudinally considerable transverse myelitis (LETM) unique from multiple sclerosis. The discovery of neuromyelitis optica immunoglobulin G (NMO-IgG) (anti-aquaporin-4 antibody) and its specificity and sensitivity for NMO has led to its incorporation in the diagnostic criteria for NMO. In addition, incomplete forms of NMO (recurrent ON, LETM) and seropositivity for NMO-IgG predicts increased risk of progression to full spectrum NMO, resulting in the designations of these conditions as neuromyelitis optica spectrum disorder (NMOSD) [3]. In this article, we present a patient with ON who was found to have APG-115 both main SS and NMO-IgG seropositivity, suggestive of NMOSD and review the recent literature concerning the overlap between main SS and NMOSD. Case presentation A 56-year-old Chinese woman with no previous history of ON, myelitis or comorbidities, complained of five days of right-sided headache and progressive visual loss initially only affecting the right eye but involving the left around the fifth day. The best-corrected visual acuity of her right eye at presentation was light belief and 6/7.5 around the left. There was right relative afferent APG-115 pupillary defect with right disc swelling. The left disc was normal. Automated perimetry (24C2 threshold, Swedish Interactive Threshold Algorithm-standard strategy, Zeiss Carl Opthalmic Systems-Humphrey Division, Dublin, CA ) could not be done in the patients right vision. The test in her left eye was unfavorable for any visual field defects (reliability indices-fixation losses: seven out of 16, false positive errors: 12%, false negative errors: 6%). A detailed ophthalmic examination was performed which included slit Mouse monoclonal antibody to Pyruvate Dehydrogenase. The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzymecomplex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), andprovides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDHcomplex is composed of multiple copies of three enzymatic components: pyruvatedehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase(E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodesthe E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of thePDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alphadeficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encodingdifferent isoforms have been found for this gene lamp examination of the anterior segment, dilated fundal examination, intraocular pressure measurement as well as fluorescein staining for corneal abnormalities. The ocular examination was APG-115 unfavorable for punctate epithelial erosions, uveitis, pars planitis or retinal vasculitis. There was no clinical evidence of transverse myelitis, and the patient declined spinal imaging due to financial constraints. Serological investigations revealed raised levels of the following autoimmune antibodies: anti-nuclear antibody, rheumatoid factor, anti-Ro antibody as well as NMO-IgG antibody. Neuroimaging showed swelling, edema and enhancement of the orbital segment of the right optic nerve with marked enhancement and stranding in the intraconal excess fat. There was also enhancement of the left optic sheath and intraconal excess fat posterior to the globe. No periventricular white matter plaques were found (Physique ?(Figure1).1). A spinal tap showed normal levels of protein and inflammatory cells and oligoclonal bands were absent. Open in a separate window Physique 1 Magnetic resonance imaging. (a) Coronal post-gadolinium fat-saturated T1-weighted image shows enhancement and stranding in the intraconal fat bilaterally, worse on the right. The right optic nerve enhances (arrow head) along with enhancement of the optic sheath. Left perineural enhancement posterior to the globe is also present, as indicated by the arrow. (b)Axial post-gadolinium fat-saturated T1-weighted image demonstrated the marked enhancement in the right intraconal fat. The patient.