Rare Immunology News

Disease Profile

CANOMAD syndrome

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

<1 / 1 000 000

US Estimated

Europe Estimated

Age of onset






Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Chronic Ataxic Neuropathy Ophthalmoplegia M-protein Agglutination Disialosyl antibodies syndrome; Chronic sensory ataxic neuropathy with anti-disialosyl antibodies


Nervous System Diseases


CANOMAD syndrome is a rare chronic immune-mediated demyelinating polyneuropathy. CANOMAD stands for Chronic Ataxic Neuropathy Ophthalmoplegia IgM paraprotein Cold Agglutinins Disialosyl antibodies. Signs and symptoms of CANOMAD may include loss of muscle, tendon, and joint sensation, abnormal gait (walk), ataxia, tingling sensation on the skin around the mouth or extremities, paralysis of eye muscles, difficulty swallowing and speaking, and rarely respiratory muscle weakness. This condition is caused by the presence of anti-diasialosyl antibodies in the body. Oral or intravenous corticosteroids, ß-interferons, plasma exchange, intravenous immunoglobulin (IVIG), and cytotoxic drugs have all been used in treating CANOMAD with varying success.[1][2][3][4][5][6]


Willison et al. (2001) described the signs, symptoms, and laboratory findings of 18 people with CANOMAD syndrome. This is the largest case series reported to date. The most prominent symptom experienced by the patients was the loss of kinesthesia. Kinesthesia refers to the perception of one's own body parts, weight, and movement. A loss of kinesthesia refers to the loss of sensation produced in muscles, tendons and joints involved in movement. These patients presented with a distinct manner of walking (gait) and an inability to coordinate voluntary muscular movements in the upper limbs. This inability to coordinate muscle activity is called 'ataxia'. These patients also experienced the feeling or sensation of pricking, tingling, or creeping on the skin in the tissues around the mouth (perioral) or extremities (acral).[1]

The majority of the cases studied (16 out of the 18) also had motor and sensory cranial nerve involvement, causing paralysis of some of the eye muscles (ophthalmoplegia), difficulty swallowing, difficulty articulating words and, rarely, respiratory muscle weakness. These symptoms were constant in some people, but for others the symptoms came and went.[1] 

In general, CANOMAD syndrome tends to have a chronic course that often extends over decades.[5]


The underlying cause of CANOMAD syndrome is poorly understood. It appears as if IgM antibodies play a crucial role in the development of the disorder.[7] It also appears as if the condition is caused in some way by the presence of anti-diasialosyl antibodies in the body. The source of anti-diasialosyl antibodies found in individuals with CANOMAD syndrome is unknown.[1]


Oral or intravenous corticosteroids, ß-interferons, intravenous immunoglobulin (IVIG), plasma exchange, and cytotoxic drugs and a combination of these therapies have all been used in treating CANOMAD syndrome with varying success. Their use, however, has not been evaluated systematically.[1][2][3][4][5] In recent years, IVIG seems to be the preferred method of treatment.[6][7]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss CANOMAD syndrome. Click on the link to view a sample search on this topic.


  1. Willison, HJ et. al.,. The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain. 2001 Oct; 124(Pt 10):1968-77. https://brain.oxfordjournals.org/content/124/10/1968.long.
  2. Scheinfeld NS. Intravenous Immunoglobulin. Medscape Reference. February 3, 2016; https://emedicine.medscape.com/article/210367-overview.
  3. Arbogast SD, Khanna S, Koontz DW, Tomsak RL, Katirji B, Leigh RJ. Chronic ataxic neuropathy mimicking dorsal midbrain syndrome. J Neurol Neurosurg Psychiatry. 2007 Nov; 78(11):1276-7. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2117616/.
  4. Relapsing sensorimotor neuropathy with ophthalmoplegia, antidisialosyl antibodies, and extramembranous glomerulonephritis. Delval A, Stojkovic T, Vermersch P. Muscle Nerve. 2006 Feb; 33(2):274-7. https://www.ncbi.nlm.nih.gov/pubmed/?term=16258949.
  5. Fontaine B. CANOMAD syndrome. Orphanet. March 2007; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=71279.
  6. Johnson K, Malkan A, Shaffi M. Facial Involuntary Movements and Respiratory Failure in CANOMAD, Responsive to IVIG Therapy. Case Rep Med. 2015; 2015:170543. https://www.hindawi.com/journals/crim/2015/170543/.
  7. Krenn M, Keir G, Wieshmann UC. CANOMAD responding to weekly treatment with intravenous immunoglobulin (IVIg). BMJ Case Rep. 2014 Apr 10; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3987528/.

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