Jin Wook Choi MD, Presenter: Nothing to Disclose

So Young Yoo MD, Abstract Co-Author: Nothing to Disclose

Hye-Kyung Yoon MD, Abstract Co-Author: Nothing to Disclose

Ji Hye Kim MD, Abstract Co-Author: Nothing to Disclose

Brain MRI is used to assess patients with mucopolysaccharidoses(MPS), in which characteristic MR findings are well established. To our knowledge, however, previous attempts to correlate clinical severity with MR finding has never been made in large series. The aim of this study is to identify MR findings that correlate with clinical severity in the children with MPS.

41 patients(age range 2-16 years; mean 7.4 years) with different subtypes of MPS(type I, 10; type II, 26; type IIIA, 1; type IIIB, 3) who underwent MRI are included in this study. We analyzed the degree of cribriform changes of periventricular white matter, basal ganglia/thalamus and corpus callosum on T1WI, the signal change of periventricular white matter on T2WI, the degree of ventriculomegaly, the degree of brain atrophy, and cord compression. Each findings were reviewed by two radiologist and were graded as follows: 0=none, 1=mild, 2=moderate, 3=severe. We also measured the depth of cisterna magna, the depth of anterior CSF space at the craniocervical junction, and soft tissue thickness posterior to the odontoid process. Degree of clinical severity was assessed by clinician according to mental retardation, and physical performance and classified as mild, moderate, or severe. Correlation between clinical severity and findings of MRI was analyzed statistically using Spearman's rho test.

Ventriculomegaly, cribriform changes of brain parenchyma and degree of atrophy were significantly correlated with clinical severity. Cribriform changes in the corpus callosum and periventricular white matter were not significantly, but tend to be, correlated with clinical severity. Although periventricular white matter signal change, mega cisterna magna and abnormalities of craniocervical junction were characteristic findings of MR imaging, those parameters were not correlated with clinical severity.

Ventriculomegaly, cribriform changes of brain parenchyma and brain atrophy on MR image were significantly correlated with clinical severity and these MR findings can be used to differentiate clinical severity in children with MPS.

Choi, J, Yoo, S, Yoon, H, Kim, J, Brain MR Findings in Children with Mucopolysaccharidoses: Correlation with Clinical Severity.  Radiological Society of North America 2005 Scientific Assembly and Annual Meeting, November 27 - December 2, 2005 ,Chicago IL. http://archive.rsna.org/2005/4419701.html