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Genetic and Rare Diseases Information Center (GARD)

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CANOMAD syndrome


Other Names for this Disease
  • Chronic Ataxic Neuropathy Ophthalmoplegia M-protein Agglutination Disialosyl antibodies syndrome
  • Chronic sensory ataxic neuropathy with anti-disialosyl antibodies
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Your 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.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is CANOMAD syndrome?

CANOMAD syndrome is a rare chronic immune-mediated demyelinating polyneuropathy. CANOMAD stands for Chronic Ataxic Neuropathy Ophtalmoplegia 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, and cytotoxic drugs have all been used in treating CANOMAD with limited success.[1][2][3][4][5]
Last updated: 3/18/2009

Are there any medical terms that I should review prior to reading information about CANOMAD syndrome?

It might be helpful to review the following descriptions before you read information about CANOMAD syndrome since most of the information available on the condition is very technical in nature.

(C)hronic - refers to the long duration of the condition. 

(A)taxic - relates to the loss of ability to coordinate muscular movement. 

(N)europathy - describes a disease or abnormality of the nervous system, especially one affecting the cranial or spinal nerves.

(O)phthalmoplegia - refers to the paralysis of one or more of the muscles of the eye.

Ig(M) paraprotein - IgM is a protein, more specifically an antibody, that is secreted by plasma cells. Plasma cells are a pale yellow mixture of water, proteins and salts. One of the functions of plasma is to act as a carrier for blood cells, nutrients, enzymes and hormones. In CANOMAD the IgM antibody is abnormally present. The term “paraprotein” actually refers to any abnormal plasma protein associated with a disease.

Cold (A)gglutinins - antibodies that are active at cold temperatures, attaching to red blood cells and causing the red cells to bind together or 'agglutinate.'

(D)isialosyl antibodies - in this disease, "anti-disialosyl antibodies" refers to the IgM antibodies.

Last updated: 3/4/2009

Why is there such limited information on CANOMAD syndrome?

There have not been many documented cases of the condition as demonstrated by the presence of very few articles on CANOMAD syndrome in the medical literature.
Last updated: 3/4/2009

What symptoms have been observed in patients with CANOMAD syndrome?

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: 3/4/2009

What causes CANOMAD syndrome?

CANOMAD syndrome is caused 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: 3/4/2009

How might CANOMAD syndrome be treated?

Oral or intravenous corticosteroids, ß-interferons, intravenous immunoglobulin, plasma exchange, and cytotoxic drugs and a combination of these therapies have all been used in treating CANOMAD syndrome with limited success. Their use however has not been evaluated systematically.[1][2][3][4][5]
Last updated: 3/4/2009

References
  • Willison, et. al.,. The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain. 2001;
  • Scheinfeld NS, Godwin JE. Intravenous Immunoglobulin. eMedicine. 2008; http://emedicine.medscape.com/article/210367-overview. Accessed 3/4/2009.
  • 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;
  • Relapsing sensorimotor neuropathy with ophthalmoplegia, antidisialosyl antibodies, and extramembranous glomerulonephritis. Delval A, Stojkovic T, Vermersch P. Muscle Nerve. 2006 Feb;
  • CANOMAD syndrome. Orphanet. 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=71279. Accessed 3/4/2009.