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CANOMAD syndrome

MedGen UID:
419872
Concept ID:
C2931684
Disease or Syndrome
Synonyms: Chronic Ataxic Neuropathy Ophthalmoplegia M-protein Agglutination Disialosyl antibodies syndrome; Chronic sensory ataxic neuropathy with anti-disialosyl antibodies
SNOMED CT: Chronic ataxic neuropathy, ophthalmoplegia, monoclonal immunoglobulin M protein, cold agglutinin and disialosyl antibody syndrome (715624006); Chronic ataxic neuropathy, ophthalmoplegia, monoclonal IgM protein, cold agglutinins and disialosyl antibodies (715624006); CANOMAD syndrome (715624006); Chronic sensory ataxic neuropathy with anti-dyalosyl IgM antibodies (715624006); CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, monoclonal IgM protein, cold agglutinins and disialosyl antibodies) syndrome (715624006)
 
Monarch Initiative: MONDO:0019109
Orphanet: ORPHA71279

Definition

A rare chronic immune-mediated demyelinating polyneuropathy. The exact prevalence is unknown but less than 30 cases have been reported in the literature. The clinical picture comprises a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. Motor weakness affecting the oculomotor and bulbar muscles is present as either a fixed or relapsing-remitting feature. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCANOMAD syndrome
Follow this link to review classifications for CANOMAD syndrome in Orphanet.

Recent clinical studies

Etiology

Dammacco R, Lisch W, Kivelä TT, Terpos E, Kastritis E, Sisto D, Mavilio A, Ria R, Alessio G, Vacca A, Dammacco F
Ocul Immunol Inflamm 2022 Oct-Nov;30(7-8):1659-1668. Epub 2021 Jul 16 doi: 10.1080/09273948.2021.1933068. PMID: 34270382
Moshe-Lilie O, Ensrud E, Ragole T, Nizar C, Dimitrova D, Karam C
BMC Neurol 2021 Feb 28;21(1):94. doi: 10.1186/s12883-021-02118-7. PMID: 33639867Free PMC Article
Kapoor M, Spillane J, Englezou C, Sarri-Gonzalez S, Bell R, Rossor A, Manji H, Reilly MM, Lunn MP, Carr A
Neurology 2020 Feb 11;94(6):e635-e638. Epub 2019 Dec 18 doi: 10.1212/WNL.0000000000008742. PMID: 31852814Free PMC Article
Caudie C, Quittard Pinon A, Bouhour F, Vial C, Garnier L, Fabien N
Clin Lab 2013;59(11-12):1277-87. doi: 10.7754/clin.lab.2013.121116. PMID: 24409662

Diagnosis

Traub R, Qarni T, Cohen AD, Karam C
Muscle Nerve 2024 Aug;70(2):173-179. Epub 2024 May 30 doi: 10.1002/mus.28164. PMID: 38816958
Moshe-Lilie O, Ensrud E, Ragole T, Nizar C, Dimitrova D, Karam C
BMC Neurol 2021 Feb 28;21(1):94. doi: 10.1186/s12883-021-02118-7. PMID: 33639867Free PMC Article
Vallat JM, Duchesne M, Corcia P, Richard L, Ghorab K, Magy L, Mathis S
Neurology 2021 Feb 2;96(5):214-225. Epub 2020 Dec 4 doi: 10.1212/WNL.0000000000011324. PMID: 33277411
McKelvie PA, Gates PC, Day T
Muscle Nerve 2013 Oct;48(4):599-603. Epub 2013 Jun 26 doi: 10.1002/mus.23897. PMID: 23649749
Kam C, Balaratnam MS, Purves A, Mills KR, Riordan-Eva P, Pollock S, Bodi I, Munro NA, Bennett DL
Muscle Nerve 2011 Nov;44(5):829-33. doi: 10.1002/mus.22167. PMID: 22006700

Therapy

Kapoor M, Spillane J, Englezou C, Sarri-Gonzalez S, Bell R, Rossor A, Manji H, Reilly MM, Lunn MP, Carr A
Neurology 2020 Feb 11;94(6):e635-e638. Epub 2019 Dec 18 doi: 10.1212/WNL.0000000000008742. PMID: 31852814Free PMC Article
Krenn M, Keir G, Wieshmann UC
BMJ Case Rep 2014 Apr 10;2014 doi: 10.1136/bcr-2013-202545. PMID: 24722712Free PMC Article
Kam C, Balaratnam MS, Purves A, Mills KR, Riordan-Eva P, Pollock S, Bodi I, Munro NA, Bennett DL
Muscle Nerve 2011 Nov;44(5):829-33. doi: 10.1002/mus.22167. PMID: 22006700
Sanvito L, Rajabally YA
Muscle Nerve 2011 Sep;44(3):451-5. doi: 10.1002/mus.22157. PMID: 21996808

Prognosis

Salamon A , Dézsi L , Radics B , Varga ET , Hortobágyi T , Tömösvári A , Vécsei L , Klivényi P , Rajda C
Ideggyogy Sz 2020 Mar 30;73(3-4):141-144. doi: 10.18071/isz.73.0141. PMID: 32364342
Kapoor M, Spillane J, Englezou C, Sarri-Gonzalez S, Bell R, Rossor A, Manji H, Reilly MM, Lunn MP, Carr A
Neurology 2020 Feb 11;94(6):e635-e638. Epub 2019 Dec 18 doi: 10.1212/WNL.0000000000008742. PMID: 31852814Free PMC Article
Krenn M, Keir G, Wieshmann UC
BMJ Case Rep 2014 Apr 10;2014 doi: 10.1136/bcr-2013-202545. PMID: 24722712Free PMC Article
McKelvie PA, Gates PC, Day T
Muscle Nerve 2013 Oct;48(4):599-603. Epub 2013 Jun 26 doi: 10.1002/mus.23897. PMID: 23649749

Clinical prediction guides

Dammacco R, Lisch W, Kivelä TT, Terpos E, Kastritis E, Sisto D, Mavilio A, Ria R, Alessio G, Vacca A, Dammacco F
Ocul Immunol Inflamm 2022 Oct-Nov;30(7-8):1659-1668. Epub 2021 Jul 16 doi: 10.1080/09273948.2021.1933068. PMID: 34270382
Caudie C, Quittard Pinon A, Bouhour F, Vial C, Garnier L, Fabien N
Clin Lab 2013;59(11-12):1277-87. doi: 10.7754/clin.lab.2013.121116. PMID: 24409662

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