MRI in the mild type of mucopolysaccharidosis II (Hunter's syndrome)
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N Shinomiya1, T Nagayama, Y Fujioka
12nd Department of Paediatrics, Ohashi Hospital, Toho University School of Medicine, Tokyo, Japan.
Neuroradiology
|July 1, 1996
Abstract:
We report imaging findings in a 3-year-old boy with the typical mild type of Hunter's disease. MRI revealed multifocal large cyst- or spindle-like areas of increased and decreased signal in the white matter, including the corpus callosum on T1- and T2-weighted images. The white matter showed high signal on T2-weighted images, isointense with cerebrospinal fluid on all other pulse sequences. To our knowledge, these appearances have not been reported in this disorder. Deposition of mucopolysaccharide and/or glycolipid and increase in fluid content seem to be responsible.