Magnetic resonance imaging of the brain in XXXX : a review of the literature and a report of four new cases with attention to the basal ganglia and imaging technique.

@entity1 @entity66 @entity13962 @entity1155 @entity62 @entity130
RATIONALE AND OBJECTIVES: In @entity13962 ( @entity13962 ), a pediatric @entity66 that may be mistaken for nonaccidental @entity130 , expeditious detection is critical as early treatment may substantially improve @entity1155 . In this study, we examine in depth the magnetic resonance (MR) findings, with special attention to the basal ganglia, in 4 new cases and compare the findings with those described in the literature. METHODS: MR studies of 4 @entity1 , diagnosed to have @entity13962 via cultured fibroblast enzyme studies or urine metabolite assays, were performed on a 1.5 T system in the axial plane using spin echo T(1)-weighted, fast spin echo T(2)-weighted, and fluid-attenuated inversion recovery (FLAIR) technique. Three of 4 @entity1 were followed with serial exams to document temporal evolution of the disease. RESULTS: On T(2)-weighted images, abnormal increased signal intensity was seen in both the putamen and globus pallidus in all cases. However, in contradistinction to cases reported in the literature, involvement of the caudate nucleus was minimal or absent even on serial MR exams. In @entity1 15 months and older, FLAIR improved recognition of basal ganglia and @entity62 . The previously described widened cerebrospinal fluid spaces anterior to the temporal lobes, increased T(2)-weighted signal intensity in the periventricular white matter, and widened sylvian fissures characteristic of @entity13962 were noted in all @entity1 . CONCLUSIONS: Abnormalities of the caudate nucleus are not a prominent presentation of these @entity1 and the absence of this finding should not exclude a diagnosis of @entity13962 . FLAIR scans, as an adjunct to more conventional T(1)- and T(2)-weighted sequences, can play an important role in @entity1 15 months or older despite immature myelination in these @entity1 .
