Clinical phenotype of patients with neuropathy associated with monoclonal gammopathy: a comparative study and a review of the literature.
The objective of this study was to investigate if the clinical and electrophysiological phenotype of patients with polyneuropathy associated with IgM monoclonal gammopathy (IgM-PNP) is related to the presence of antibodies against gangliosides or myelin-associated glycoprotein (MAG). We compared clinical and nerve conduction study (NCS) characteristics of 11 IgM-PNP patients with antibodies against asialo-GM1 or gangliosides (GM1, GD1a, GD1b, GM2 or GQ1b) to 11 consecutive IgM-PNP patients with anti-MAG neuropathy and to 9 IgM-PNP patients without antibodies against either MAG or gangliosides. Patients with anti-ganglioside antibodies could not be differentiated from those with anti-MAG antibodies based on clinical characteristics. However, within the group of anti-ganglioside antibody positive patients, antibodies against GD1b and GQ1b were associated with a purely sensory neuropathy (p = 0.002), while asymmetric weakness with symmetric sensory loss was associated with anti-asialo-GM1 antibodies. In conclusion, polyneuropathy associated with IgM monoclonal gammopathy and anti-ganglioside antibodies clinically resembles anti-MAG neuropathy. Pure sensory neuropathy and marked asymmetry may suggest the presence of anti-ganglioside rather than anti-MAG antibodies.
Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center Utrecht, Heidelberglaan 100, PO Box 85500 3508 GA, 3584 CX, Utrecht, The Netherlands, astork2@umcutrecht.nl.
Abraham C J
AC
Stork
W-Ludo
WL
van der Pol
Hessel
H
Franssen
Bart C
BC
Jacobs
Nicolette C
NC
Notermans
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004185:Disability Evaluation; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology; D008297:Male; D008875:Middle Aged; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D009431:Neural Conduction / Q000502:physiology; D010265:Paraproteinemias / Q000150:complications*; D010523:Peripheral Nervous System Diseases / Q000150:complications*; D010641:Phenotype; D012189:Retrospective Studies; D018709:Statistics, Nonparametric
D005732:Gangliosides; D007075:Immunoglobulin M; C115082:MAG protein, human; D018992:Myelin-Associated Glycoprotein