Clinical features
There are a few clinical presentations, depending on time of onset. Features include:
- hepatosplenomegally
- inguinal and umbilical herniae
- coarse facial features - as in Hurler's syndrome
- deafness
- dysphagia
- blindness
- cherry red spot on the macula
- dysostosis multiplex
The late onset form may present with dysarthria and ataxia.
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