ArticleAJNR. American journal of neuroradiology2012
Closed Meningo(encephalo)cele: a new feature in Hunter syndrome.
Article in AJNR. American journal of neuroradiology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 13 citations in OpenAlex.
- Targeting Neurological Aspects of Mucopolysaccharidosis Type II: Enzyme Replacement Therapy and Beyond.BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2024Review
- Brain Proteomic Profiling in Intractable Epilepsy Caused by TSC1 Truncating Mutations: A Small Sample Study.Frontiers in neurology · 2020Article
- Mucopolysaccharidoses: overview of neuroimaging manifestations.Pediatric radiology · 2018Review
- Chiari 1 malformation and holocord syringomyelia in hunter syndrome.JIMD reports · 2014Article
- Imaging findings of mucopolysaccharidoses: a pictorial review.Insights into imaging · 2013Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeHunter syndrome (MPS type II) is a rare X-linked recessive disease caused by lysosomal enzyme iduronate-2-sulfatase deficiency, characterized by frequent and variable brain and skull involvement. Our objective was determine the frequency of closed cephaloceles in a large cohort of subjects affected with Hunter syndrome and to investigate possible correlations with clinical and neuroradiologic findings. MATERIALS AND
methodsBrain MR imaging of 33 patients (32 males and 1 female, age range 2.5-30.8 years, mean age 10.4 years) affected with Hunter syndrome were retrospectively evaluated. Eleven (age range 3.6-30.8 years; mean age 15.1) presented with an "attenuated" phenotype, while 22 (age range 2.5-19.1 years; mean age 8.2) had a "severe" phenotype.
resultsA closed cephalocele was detected in 9/33 patients (27%) at the level of anterior and middle fossa in 6 and 3 cases, respectively; 6/9 subjects were affected with the attenuated phenotype and 1/9 suffered from epilepsy. Closed cephaloceles did not show a significant association with other brain and spine MR imaging features of Hunter disease, such as enlargement of perivascular spaces, cisterna magna, pituitary sella, ventricles and subarachnoid spaces, craniosynostosis, dens hypoplasia, white matter abnormalities, spinal stenosis due to periodontoid cap, platyspondylia, or intervertebral disk anomalies.
conclusionsClosed cephaloceles are frequent in Hunter syndrome and should be considered a neuroradiologic feature of this disease.
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