MRI findings in children with vanishing white matter disease / 中华放射学杂志

ABSTRACT

Objective To describe the MRI features of vanishing white matter disease (VWM).Methods Ten patients diagnosed as VWM between 2008-2013 were enrolled,with the mean age of(40 ±23)months.The clinical materials and MR images of them were reviewed retrospectively.The findings of MRI were analyzed by two experienced neuroradiologists in this study,including the location (cerebrum,cerebellum,brainstem,corpus callosum and internal/external capsule),size and signal intensity of the lesions,as well as the presence of cystic degeneration,cerebrum atrophy and the abnormal signal of the basal nuclei.Results MRI of brain typically showed extensive abnormal signal in the cerebral central white matter (10/10) and the focal lesion in subcortical white matter (9/10).The involvement of U-fibers was present in 7 cases.Cystic degeneration of the affected white matter was found in 7 cases.Abnormal signal also appeared in the white matter of posterior limb of the internal capsule (8/10),the external capsule (7/10),the inner rim (the callosal-septal interface) of the corpus callosum (8/10).The lesions of the thalamus and globus pallidus were detected in 2 cases.The white matter of the brainstem and cerebellum were involved in 6 and 7 cases respectively.Conclusion VWM typically shows a diffuse and symmetrical abnormal signal in the cerebral white matter with cystic degeneration on MRI,which is usually diagnostic.