National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

CANOMAD syndrome


Other Names:
Chronic Ataxic Neuropathy Ophthalmoplegia M-protein Agglutination Disialosyl antibodies syndrome; Chronic sensory ataxic neuropathy with anti-disialosyl antibodies
Categories:
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]
Last updated: 4/18/2016
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]

Last updated: 4/18/2016
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]
Last updated: 4/18/2016
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]
Last updated: 4/18/2016

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes other inflammatory peripheral neuropathies, particularly chronic inflammatory demyelinating polyneuropathy, as well as vascular and demyelinating brainstem lesions.
Visit the Orphanet disease page for more information.

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.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I have had a very hard time finding information on CANOMAD. A loved one has had this condition for 4 years. I would like to learn more about it. See answer


  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. http://brain.oxfordjournals.org/content/124/10/1968.long.
  2. Scheinfeld NS. Intravenous Immunoglobulin. Medscape Reference. February 3, 2016; http://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; http://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. http://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/.