Rare types of diabetes are a very small percentage. Wolfram syndrome is one of them. Would you like to know more?

Apart from the most well-known types of diabetes (type 1, type 2, and gestational) there are others that represent a very small percentage and are rare. Among these cases is Wolfram Syndrome. This disease has blindness and type 1 diabetes as its principal symptoms. Do you want to know more about it?

What is Wolfram Syndrome?

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Wolfram Syndrome (WS) is a rare disease that only a small part of the population has. This condition is neurodegenerative and affects multiple systems of the body. It is also known as DIDMOAD for the acronym of its symptoms: diabetes insipidus, diabetes mellitus, optic atrophy and deafness.

It is a gradually aggravating disease. For this reason, people who suffer from it progressively deteriorate with time. The life expectancy of people with this condition is not longer than 50 years.

The causes of this syndrome are unknown. There are very few cases in which this condition has developed without having a family history. But, usually, genetics plays a major role. To be born with this disease, both parents must have the wolframine gene altered. This is extremely rare.

Usually, the parents are healthy carriers. This means that the disease has not developed in them. So, they do not know that they have the genetic alteration and that they will transmit it.

Symptoms of Wolfram Syndrome

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The symptoms of Wolfram Syndrome are gradually increasing. In fact, they also become more severe over time. Some of them are:

  • Type 1 diabetes.
  • Blindness due to damage to the nerves linking the eyes and the brain (optic atrophy).
  • Changes in the inner ear (deafness).
  • Severe thirst.
  • Imbalance of body fluids (diabetes insipidus).
  • Balance and coordination problems.
  • Loss of smell (anosmia).
  • Muscle spasms.
  • Insomnia.
  • Psychiatric disorders.

Normally, the first symptom to present is type 1 diabetes. It usually happens when the child is less than 6 years old. To control it, it is very common that they need insulin. This occurs in virtually all people who have Wolfram Syndrome. If you have this syndrome Cori can be very helpful for the control of your diabetes. Would you like to try it?


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Optic atrophy appears afterwards. Around the age of 11, the child begins to lose visual acuity and peripheral vision. Also, he/she begins to have difficulty visioning colors correctly (dyschromatopsia). As a general rule, over time, people with this syndrome usually lose all of their vision.

The following thing to deteriorate is usually the hearing. As with the rest of the symptoms, it happens little by little until the patient completely loses his or her ability to hear.

Around the age of 20 is when the symptoms of diabetes insipidus begin. Urinary incontinence and infections become a daily event.

The last symptoms to appear are the neurological ones. Loss of balance and coordination (ataxia), lack of smell (anosmia) or muscle spasms are the common ones. They may also have sleeping problems or psychiatric disorders.

Can Wolfram syndrome be prevented?

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No. Since it is a mutation, there is no way to prevent this syndrome. Additionally it has no cure. People with this condition are treated for each one of the symptoms.


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