Willison H J, Paterson G, Veitch J, Inglis G, Barnett S C
Department of Neurology, Southern General Hospital, Glasgow, United Kingdom.
J Neurol Neurosurg Psychiatry. 1993 Nov;56(11):1178-83. doi: 10.1136/jnnp.56.11.1178.
A patient with a chronic, large fibre sensory neuropathy had an immunoglobulin M lambda monoclonal paraprotein reactive at titres in excess of 1/10(5) with NeuNAc(alpha 2-8)NeuNAc(alpha 2-3)Gal configured disialosyl groups present on the gangliosides GD1b, GT1b, GQ1b, and GD3. The paraprotein showed weaker reactivity with GD1a, GM3, and LM1 but no reactivity with GM2, GM1, or asialo-GM1. In addition, the paraprotein had cold agglutinating activity with anti-Pr2 specificity, Pr2 being an antigenic determinant on membrane glycoproteins or glycolipids in erythrocytes or both. A large fibre sensory neuropathy with monoclonal anti-disialosyl antibodies is an increasingly recognised form of paraproteinaemic neuropathy.
一名患有慢性大纤维感觉神经病变的患者，其免疫球蛋白Mλ单克隆副蛋白与神经节苷脂GD1b、GT1b、GQ1b和GD3上存在的NeuNAc(α2-8)NeuNAc(α2-3)Gal构型的二唾液酸基团反应，滴度超过1/10(5)。该副蛋白与GD1a、GM3和LM1的反应较弱，但与GM2、GM1或脱唾液酸GM1无反应。此外，该副蛋白具有抗Pr2特异性的冷凝集活性，Pr2是红细胞膜糖蛋白或糖脂或两者上的抗原决定簇。伴有单克隆抗二唾液酸抗体的大纤维感觉神经病变是一种越来越被认可的副蛋白血症性神经病变形式。
