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Posterior Fossa Horns in Hurler Syndrome: Prevalence and Regression
AJNR. American Journal of Neuroradiology
|July 6, 2023
Summary
Posterior fossa horns, a cranial abnormality in Hurler Syndrome, were present in most patients. These horns showed natural regression with age, often before treatment, suggesting unknown skull development effects.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- Posterior fossa horns are recently identified cranial abnormalities in mucopolysaccharidoses, particularly Hurler Syndrome.
- The development and natural history of these horns are not well-understood.
Purpose of the Study:
- To investigate the prevalence, characteristics, and natural history of posterior fossa horns in patients with mucopolysaccharidosis I-Hurler syndrome.
- To describe the regression patterns of posterior fossa horns in relation to age and treatment.
Main Methods:
- Retrospective analysis of 286 brain MR imaging studies from 61 patients with mucopolysaccharidosis I-Hurler syndrome.
- Measurement of posterior fossa horn height and observation of changes over time.
Main Results:
- Posterior fossa horns were observed in 93.4% of patients.
- Initial average horn heights were 4.5 mm (right) and 4.7 mm (left).
- Most horns regressed with age, frequently before transplantation, a trend not previously described.
Conclusions:
- Posterior fossa horns are a common finding in Hurler Syndrome.
- The natural regression of these horns with age, often preceding treatment, suggests novel insights into skull development in mucopolysaccharidosis.
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