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[Identification and characterization of a monoclonal IgM reacting with disialylated gangliosides recognizing the CANOMAD syndrome].
Ann Biol Clin (Paris). 2011 Jul-Aug; 69(4):476-80.AB

Abstract

We reported the laboratory phenotype of a monoclonal IgM-lambda against disialylated gangliosides, in a 81-year-old man admitted to a neurological department because of the progressive development of distal paresthesias, gait unsteadiness, difficulty to walk and having falls. Serological studies revealed an IgM monoclonal protein with lambda light chain component of MGUS type. IgM level was 4 g/L. The positive laboratory studies showed high titers of IgM antibodies in excess of 1/10(5) against specific disialylated gangliosides including GD1b, GD3, GT1b and GQ1b. There was no serum IgM binding to MAG and SGPG/SGLPG. Clonality by in-house immunodot of ganglioside antibodies was demonstrated using kappa and lambda light chain specific antibodies. Light chain subtype of the anti-ganglioside antibody activity and monoclonal IgM was lambda subtype. The reactivity at high titers was against gangliosides containing the disialosyl epitope. The clinical and laboratory features have been described under the acronym CANOMAD: Chronic Ataxic Neuropathy with Ophthalmoplegia, M proteins, cold Agglutinins and Disialosyl antibodies. Administration of IVIg produced a significant neurological improvement during six years. Then the neuropathy became refractory in the IVIg and worsened in severity, a cure by Rituximab® was established. The patient died from a pneumopathy only two months later. Monoclonal IgM binding to disialylated gangliosides have high level of specificity for diagnosis of the CANOMAD syndrome.

Authors+Show Affiliations

Centre de biologie et pathologie Est, Service de neurobiologie, Groupement hospitalier Est, Lyon Bron.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

21896416

Citation

Boussaïd, Ismael, et al. "[Identification and Characterization of a Monoclonal IgM Reacting With Disialylated Gangliosides Recognizing the CANOMAD Syndrome]." Annales De Biologie Clinique, vol. 69, no. 4, 2011, pp. 476-80.
Boussaïd I, Bouhour F, Vial C, et al. [Identification and characterization of a monoclonal IgM reacting with disialylated gangliosides recognizing the CANOMAD syndrome]. Ann Biol Clin (Paris). 2011;69(4):476-80.
Boussaïd, I., Bouhour, F., Vial, C., & Caudie, C. (2011). [Identification and characterization of a monoclonal IgM reacting with disialylated gangliosides recognizing the CANOMAD syndrome]. Annales De Biologie Clinique, 69(4), 476-80. https://doi.org/10.1684/abc.2011.0603
Boussaïd I, et al. [Identification and Characterization of a Monoclonal IgM Reacting With Disialylated Gangliosides Recognizing the CANOMAD Syndrome]. Ann Biol Clin (Paris). 2011 Jul-Aug;69(4):476-80. PubMed PMID: 21896416.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Identification and characterization of a monoclonal IgM reacting with disialylated gangliosides recognizing the CANOMAD syndrome]. AU - Boussaïd,Ismael, AU - Bouhour,Françoise, AU - Vial,Christophe, AU - Caudie,Christiane, PY - 2011/9/8/entrez PY - 2011/9/8/pubmed PY - 2011/11/5/medline SP - 476 EP - 80 JF - Annales de biologie clinique JO - Ann Biol Clin (Paris) VL - 69 IS - 4 N2 - We reported the laboratory phenotype of a monoclonal IgM-lambda against disialylated gangliosides, in a 81-year-old man admitted to a neurological department because of the progressive development of distal paresthesias, gait unsteadiness, difficulty to walk and having falls. Serological studies revealed an IgM monoclonal protein with lambda light chain component of MGUS type. IgM level was 4 g/L. The positive laboratory studies showed high titers of IgM antibodies in excess of 1/10(5) against specific disialylated gangliosides including GD1b, GD3, GT1b and GQ1b. There was no serum IgM binding to MAG and SGPG/SGLPG. Clonality by in-house immunodot of ganglioside antibodies was demonstrated using kappa and lambda light chain specific antibodies. Light chain subtype of the anti-ganglioside antibody activity and monoclonal IgM was lambda subtype. The reactivity at high titers was against gangliosides containing the disialosyl epitope. The clinical and laboratory features have been described under the acronym CANOMAD: Chronic Ataxic Neuropathy with Ophthalmoplegia, M proteins, cold Agglutinins and Disialosyl antibodies. Administration of IVIg produced a significant neurological improvement during six years. Then the neuropathy became refractory in the IVIg and worsened in severity, a cure by Rituximab® was established. The patient died from a pneumopathy only two months later. Monoclonal IgM binding to disialylated gangliosides have high level of specificity for diagnosis of the CANOMAD syndrome. SN - 0003-3898 UR - https://www.unboundmedicine.com/medline/citation/21896416/[Identification_and_characterization_of_a_monoclonal_IgM_reacting_with_disialylated_gangliosides_recognizing_the_CANOMAD_syndrome]_ L2 - http://www.jle.com/medline.md?issn=0003-3898&vol=69&iss=4&page=476 DB - PRIME DP - Unbound Medicine ER -