MPS II - Hunter

hunterHunter Syndrome is also known as MPS II. It takes its name from Charles Hunter, the professor of Medicine in Manitoba, Canada who first described two brothers with the disorder in 1917. MPS II is different from the other MPS disorders in that it generally affects males only, except in very rare cases.

MPS II covers a wide spectrum of severity of symptoms. All MPS II individuals lack the same enzyme called iduronate sulphate sulphatase. The gene responsible has been located and it is often possible to analyse the patient's DNA and predict how severe the syndrome may be. To some extent the age of diagnosis reflects the severity and indicates the future progress that can be expected. However, MPS II is generally a severe physical disability for all affected individuals, regardless of whether they are mildly or more severely affected.

Those with normal or near normal intelligence are said to have mild Hunter Syndrome. However, the severe form of Hunter Syndrome is characterised by gradual deterioration of both the brain and body.

It is important to note that each family will have its own particular mutation which will affect the severity of the condition and its development. The latest understanding is that some people seem to produce some enzyme activity which helps to slow down the progression of the disorder whilst those with more severe symptoms appear to have no enzyme activity (or function) at all.

There is a very wide range of life expectancy according to the severity of the disorder. Those who are mildly affected may have a reasonably normal life span if their physical problems are not severe. There is a report of one Hunter patient living to 87 years. Sadly, those who are severely affected are likely to die before reaching their mid-teens and some may die much earlier. Death results from complications associated with the disorder such as heart disease or respiratory problems.

The Australian prevalence of Hunter syndrome is 1 in 136,000.

More information on MPS II is available from our National Office, including an MPS II syndrome booklet which can be purchased with the enclosed order form.