ABSTRACT
A 65-year-old woman was referred for sudden bilateral loss of vision. She was vaccinated a few days earlier. The ophthalmological examination showed a massive optic disc swelling on both eyes. Magnetic resonance imaging (MRI) revealed suspected bilateral optic neuritis without cerebral involvement or transverse myelitis. After serological detection of anti-MOG (myelin oligodendrocyte glycoprotein) antibodies, the patient was treated with high-dose corticosteroid pulse therapy until vision recovered. Discussion: an atypical optic neuritis may indicate a neuromyelitis optica spectrum disorder (NMOSD), which should be further characterized by determination of Aquaporin 4(AQP4)-IgG and MOG-IgG.
Subject(s)
Neuromyelitis Optica , Optic Neuritis , Aged , Aquaporin 4 , Female , Humans , Magnetic Resonance Imaging , Myelin-Oligodendrocyte Glycoprotein/metabolism , Neuromyelitis Optica/diagnosis , Neuromyelitis Optica/drug therapy , Optic Neuritis/diagnosis , Optic Neuritis/drug therapyABSTRACT
CINCA syndrome is an autoinflammatory disease in childhood characterized by multisystemic manifestations: cutaneous, articular, and neurological including sensory organs. We report the case of homozygous twins affected by CINCA syndrome. The diagnosis was evoked on the basis of multiple systemic symptoms (multiple episodes of fever of unknown origin, mental retardation, short stature, meningitis, hearing loss, bilateral papilledema) and confirmed by the presence of a CIAS1 mutation on genetic analysis. After few months of treatment by anakinra (an interleukin-1 receptor antagonist) the children began to grow again and we noted regression of the biological inflammatory syndrome.
Subject(s)
Cryopyrin-Associated Periodic Syndromes/complications , Diseases in Twins/complications , Papilledema/etiology , Child , Humans , MaleABSTRACT
The αGal HyperAcute(®) Technology exploits a robust zoonotic blockade to enhance potency of antiviral vaccines. Naturally acquired immunity against the common αGal epitope [galactose-alpha(1,3)-galactose-beta(1,4)N-acetylglucosamine-R (Gal-α(1,3)-Gal-ß(1,4)-GlcNAc-R)] is facilitated by the loss of a key enzyme in the epitope's biosynthetic pathway. As human cells are devoid of this epitope, chronic stimulus from gut flora leads to high levels of circulating anti-αGal antibodies and the development of a robust immune pathway. As the αGal epitope is immediately recognized as foreign, the naturally acquired αGal immune pathway in humans serves as a strong barrier to zoonotic infection. The αGal HyperAcute(®) Technology takes advantage of this natural process to facilitate the rapid presentation of modified antigens to antigen-presenting cells, leading to a strong immune response. The evolutionary immunity to αGal ensures that the presence of αGal epitopes on antigens will lead to a robust immune response involving cross-activation of T(H)1 immunity, characterized by cytokine secretion and increased phagocytic activity, and T(H)2 immunity characterized by high antibody titres. αGal epitopes can be applied to antiviral vaccines by biological, enzymatic or chemical means. Several detection methods that directly and indirectly verify αGal addition are discussed. Enhanced immunogenicity (humoral and cellular) of αGal-modified vaccines is shown for several antiviral vaccine candidates. αGal modification of antiviral vaccine components leads to enhanced immunogenicity. The existing body of literature describing the utility of αGal epitopes as a safe and robust immunostimulatory and -modulatory agent in humans supports the basis for applying the αGal HyperAcute(®) Technology to the improvement of antiviral vaccines, both new and currently approved.
