[Ataxic neuropathy associated with disialosylated antibodies: description of new clinical and biochemical forms].
BACKGROUND
Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin associated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, GD3, GT1b, GT1a, GQ1b.


METHODS
We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antibodies and for two of them cold agglutinins. Such features have been previously described under the acronym "CANOMAD" (chronic ataxic neuropathy with ophthalmoplegia, M protein, agglutination and disialosyl antibodies).


RESULTS
One of the patients presents extramembranous glomerulopathy and severe motor disability associated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous immunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus.


CONCLUSIONS
Our study demonstrates that spectrum of polyneuropathy associated with monoclonal polyneuropathy may be larger than originally described.
Delval
A
A
Service de Neurologie Générale et de Pathologie Inflammatoire, Clinique Neurologique, CHRU de Lille.
Stojkovic
T
T
de Sèze
J
J
Hurtevent
J-F
JF
Glowacki
F
F
Beaume
A
A
Destée
A
A
Vermersch
P
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000477:Alkylating Agents / Q000627:therapeutic use; D000906:Antibodies / Q000276:immunology* / Q000378:metabolism; D001259:Ataxia / Q000209:etiology / Q000276:immunology* / Q000378:metabolism; D001797:Blood Protein Electrophoresis; D001799:Blood Sedimentation; D003520:Cyclophosphamide / Q000627:therapeutic use; D004568:Electrodiagnosis; D015433:Glomerulonephritis, Membranous / Q000150:complications; D006801:Humans; D007116:Immunization, Passive; D007075:Immunoglobulin M / Q000276:immunology; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications; D019158:N-Acetylneuraminic Acid / Q000276:immunology* / Q000378:metabolism; D009431:Neural Conduction; D009886:Ophthalmoplegia / Q000209:etiology / Q000503:physiopathology; D011115:Polyneuropathies / Q000150:complications / Q000276:immunology* / Q000628:therapy
D000305:Adrenal Cortex Hormones; D000477:Alkylating Agents; D000906:Antibodies; D007075:Immunoglobulin M; D003520:Cyclophosphamide; D019158:N-Acetylneuraminic Acid