Daripa Bob, Lucchese Scott
Medicine, Grant Medical College and Sir Jamsetjee Jeejeebhoy (JJ) Group of Government Hospitals, Mumbai, IND.
Internal Medicine/Neurology, Singapore General Hospital, Singapore, SGP.
Cureus. 2022 Apr 28;14(4):e24551. doi: 10.7759/cureus.24551. eCollection 2022 Apr.
Galactose-α-1,3-galactose, an oligosaccharide epitope better acknowledged as α-Gal, is present in non-primate mammal meat, tick bites, microorganisms, and vaccines as a glycoprotein or glycolipid moiety. This can manifest hyperimmune reactions as it enters the human body, known as α-Gal syndrome (AGS). AGS and Guillain-Barré syndrome share cognate immunogenic pathomechanism via conquering immune tolerance further speculating galactose navigated neurological sequel. Unusual symptomatic presentation of abulia in our case, with incidental finding of high titers of α-Gal specific IgE immunoglobulin further supported by temporal resolution of symptoms on abstinence of meat products, raises a high degree of suspicion of neuro-psychiatric manifestation in sensitized α-Gal patients. The pathomechanism is blurry, and an absence of an objective diagnostic tool makes the neurological diagnosis challenging. α-Gal driven immune-related hypothalamic dysfunction could be a possibility that needs further exploration and is a topic of research.
半乳糖-α-1,3-半乳糖,一种更被熟知为α-Gal的寡糖表位,以糖蛋白或糖脂部分的形式存在于非灵长类哺乳动物肉、蜱虫叮咬、微生物及疫苗中。当它进入人体时会引发超敏反应,即α-Gal综合征(AGS)。AGS与吉兰-巴雷综合征通过打破免疫耐受共享同源免疫致病机制,进一步推测半乳糖会引发神经后遗症。我们病例中出现的异常无欲状症状表现,以及偶然发现的高滴度α-Gal特异性IgE免疫球蛋白,且在禁食肉类产品后症状随时间缓解,这进一步支持了致敏α-Gal患者存在神经精神表现的高度怀疑。致病机制尚不明确,且缺乏客观诊断工具使得神经学诊断具有挑战性。α-Gal驱动的免疫相关下丘脑功能障碍可能是一个需要进一步探索的可能性,也是一个研究课题。