About: Metachromatic leukodystrophy   Sponge Permalink

An Entity of Type : owl:Thing, within Data Space : 134.155.108.49:8890 associated with source dataset(s)

In infants, the disease will usually manifest between the ages of 18 & 24 months as difficulty walking, muscle wasting and weakness, developmental delays, loss of vision and convulsions eventually leading to paralysis, dementia and death generally before the first decade. In adults, the disease almost always presents as a form of mental illness. In adults, the symptoms progress very slowly and may last for more than a decade. There is no treatment for any form of the disease, so care concentrates on alleviating some of the worse symptoms.

AttributesValues
rdfs:label
  • Metachromatic leukodystrophy
rdfs:comment
  • In infants, the disease will usually manifest between the ages of 18 & 24 months as difficulty walking, muscle wasting and weakness, developmental delays, loss of vision and convulsions eventually leading to paralysis, dementia and death generally before the first decade. In adults, the disease almost always presents as a form of mental illness. In adults, the symptoms progress very slowly and may last for more than a decade. There is no treatment for any form of the disease, so care concentrates on alleviating some of the worse symptoms.
dcterms:subject
mortalityrate
  • Inevitably fatal
symptom
  • Difficulty walking, muscle wasting and weakness, appearance of mental illness
dbkwik:house/prope...iPageUsesTemplate
Name
  • Metachromatic leukodystrophy
Type
treatment
  • None
Cause
  • Inheriting faulty gene from both parents
abstract
  • In infants, the disease will usually manifest between the ages of 18 & 24 months as difficulty walking, muscle wasting and weakness, developmental delays, loss of vision and convulsions eventually leading to paralysis, dementia and death generally before the first decade. In teh juvenile from fo the disease, the disease usually starts with difficulty keeping up in school, followed by mental deterioration and dementia. The symptoms that affect younger children then present themselves, but much more slowly. However, most patients who develop the disease at this stage die in their second or third decade. In adults, the disease almost always presents as a form of mental illness. In adults, the symptoms progress very slowly and may last for more than a decade. There is no treatment for any form of the disease, so care concentrates on alleviating some of the worse symptoms. The condition is not terribly uncommon, and is very common in certain isolated groups. For example, in Israeli Jews of South Arabian extraction, it is as high as 1 in 76 people, and of Arabs living in Israel, it is 1 in 8,000. In the general population, the incidence is somewhere between 1 in 40,000 and 1 in 160,000.
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