Brain magnetic resonance imaging in 23 patients with mucopolysaccharidosesand the effect of bone marrow transplantation

Citation
T. Seto et al., Brain magnetic resonance imaging in 23 patients with mucopolysaccharidosesand the effect of bone marrow transplantation, ANN NEUROL, 50(1), 2001, pp. 79-92
Citations number
23
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
ANNALS OF NEUROLOGY
ISSN journal
0364-5134 → ACNP
Volume
50
Issue
1
Year of publication
2001
Pages
79 - 92
Database
ISI
SICI code
0364-5134(200107)50:1<79:BMRII2>2.0.ZU;2-3
Abstract
A longitudinal study of cranial magnetic resonance imaging (MRI) was carrie d out in 23 patients with mucopolysaccharidoses (MPS); 1 each of types IH, VI, and VII; 2 of type IS; 10 of type II; and 4 each of types IIIB and Nk S ix types of distinct abnormalities were 1) cribriform changes or spotty cha nges in the corpus callosum, basal ganglia, and white matter; 2) high-inten sity signal in the white matter on T2-weighted image; 3) ventriculomegaly; 4) diffuse cerebral cortical atrophy; 5) spinal cord compression; and 6) me gacisterna magna. The cribriform changes that corresponded to dilated periv ascular spaces were found in the patients with MPS IS, II, and VI. The patc hy and diffuse intensity changes were found in the patient with MPS II and IIIB, respectively. MPS IH and the severe type of MPS II showed marked vent riculomegaly. Marked cerebra atrophy was observed in all MPS IIIB patients and in the severe type of MPS II patients. Spina cord compression was a fea ture usuary observed in MPS IH, TVA, M, and VII. Megacisterna magna was fre quent in the patients with MPS II (6/10). In two of five patients, the ther apeutic effect of bone marrow transplantation (BMT) was remarkable. Both th e cribriform changes and the intensity change of the white matter in a MPS VI patient disappeared eight years after the BMT. Slight improvement of cri briform change was noted in one patient with MPS II three years after the B MT. MRS was not sufficient to estimate the accumulation of glycosaminoglyca ns but was useful for evaluating neuronal damages.