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BackgroundAnti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein–Barr virus (EBV) infection.Case presentationA 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection. The MRI revealed the lesions in the cervical spinal cord, the conus medullaris, and the internal capsule. An examination of the cerebrospinal fluid revealed pleocytosis. Cell-based immunoassays revealed positivity for anti-MOG antibody with a titer of 1:1024 and negativity for anti-aquaporin-4 antibody. His symptoms quickly improved after steroid pulse therapy followed by oral betamethasone. Anti-MOG antibody titer at the 6-month follow-up was negative.ConclusionsThis case suggests that primary EBV infection would trigger anti-MOG antibody-positive ADEM. Adult ADEM patients can be positive for anti-MOG antibody, the titers of which correlate well with the neurological symptoms.

GISTs express KIT (CD117), a receptor tyrosine kinase encoded by protooncogene c-kit. Most of the sporadic GIST cases have somatic gain-of-function mutations of the c-kit gene mostly at exon 11, followed by exons 9, 13 and 17.(3-5) Moreover, a minor subset (approximately 5-7%) of GIST exhibits activating mutations of platelet-derived growth factor receptor-alpha (PDGFRα) at exons 12, 14 and 18. (5,6) In the recent past, molecular targeting treatments with imatinib mesylate (STI571), which is a selective inhibitor of ABL (BCR-ABL), KIT, and PDGFR tyrosine kinases, were used in patients with KITpositive GIST, with significant effectiveness against metastatic and unresectable GISTs. (7,8) The antitumor activity of imatinib is heterogeneous, depending on the type of KIT mutation; tumors with KIT exon 11 mutation are more likely to respond to imatinib, while tumors with wild-type KIT or those with other types of mutations are less responsive to imatinib.(9,10) Thus, further development of new drugs is preferable. Although GISTs exhibit a spectrum of malignant potential behavior, the molecular mechanisms of tumor progression, growth, and invasion have not been fully elucidated. Studies on these mechanisms should provide opportunities to design new molecular therapeutic targets.Metalloproteinases, such as matrix metalloproteinases (MMPs) and a disintegrin and metalloproteinases (ADAMs) are implicated in various normal and pathophysiological conditions, such as fetal development, cancer development and progression, and inflammatory responses, through proteolysis. Most MMPs are secreted extracellularly except for membrane type (MT)-MMPs, and play a role in tumor biology predominantly by enhancing proteolysis of extracellular matrix macromolecules. On the other hand, ADAMs are a family of genes of multidomain membrane-anchored proteins. They cleave various proteins such as precursors of growth factors and cytokines, receptors, and membrane-anchored proteins to modulate cellular functions.(11) Among ADAMs, there is evidence that ADAM17 is an important regulator of growth factor precursor shedding. Although ADAM17 was initially identified as the metalloproteinase responsible for the shedding of tumor necrosis factor α (TNF-α), (12) it is also necessary for the shedding of several epidermal growth factor receptor (EGFR) ligands including transforming growth factor α (TGF-α), heparin-binding epidermal growth factor (HB-EGF), and amphiregulin. (13,14) To our knowledge, there is little or no information on the expression of ADAMs and their roles in gastric GISTs. In the present study, we investigated the expression of ADAMs and MMPs in gastric GISTs. The results showed preferential upregulation of ADAM17 in GISTs, relative to non-neoplastic gastric tissues. The interstitial cells of Cajal in non-neoplastic tissues did not express ADAM17 immunohistochemically. Moreover, EGFR expression with its phosphorylated forms and expression of ADAM17-sensitive EGFR ligands, amphiregulin and HB-EGF, including their shed forms, were also fou...

This study confirmed a positive survival effect with NIV, which was less effective in patients with bulbar dysfunction. Additional studies are required to determine the best timing for using NIV with ALS in patients with bulbar dysfunction.

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