Hj. Willison et al., PERIPHERAL NEUROPATHY ASSOCIATED WITH MONOCLONAL IGM ANTI-PR2 COLD AGGLUTININS, Journal of Neurology, Neurosurgery and Psychiatry, 56(11), 1993, pp. 1178-1183
A patient with a chronic, large fibre sensory neuropathy had an immuno
globulin Mlambda monoclonal paraprotein reactive at titres in excess o
f 1/10(5) with NeuNAc(alpha2-8)NeuNAc(alpha2-3)Gal configured disialos
yl groups present on the gangliosides GD1b, GT1b, GQ1b, and GD3. The p
araprotein showed weaker reactivity with GD1a, GM3, and LM1 but no rea
ctivity with GM2, GM1, or asialo-GM1. In addition, the paraprotein had
cold agglutinating activity with anti-Pr2 specificity, Pr2 being an a
ntigenic determinant on membrane glycoproteins or glycolipids in eryth
rocytes or both. A large fibre sensory neuropathy with monoclonal anti
-disialosyl antibodies is an increasingly recognised form of paraprote
inaemic neuropathy.