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Transthyretin-Related Familial Amyloid Polyneuropathy: Evaluation of CSF Enhancement on Serial T1-Weighted and Fluid-Attenuated Inversion Recovery Images following Intravenous Contrast Administration

Toshinori Hiraia, Yukio Andob, Masayuki Yamuraa, Mika Kitajimaa, Yoshiko Hayashidaa, Yukunori Korogid, Taro Yamashitac and Yasuyuki Yamashitaa

a From the Departments of Diagnostic Radiology, University of Occupational and Environmental Health, School of Medicine, Kitakyushu, Japan
b Diagnostic Medicine, University of Occupational and Environmental Health, School of Medicine, Kitakyushu, Japan
c Neurology, University of Occupational and Environmental Health, School of Medicine, Kitakyushu, Japan
d Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; and the Department of Radiology, University of Occupational and Environmental Health, School of Medicine, Kitakyushu, Japan




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FIG 1. Case 3, a 52-year-old woman with Tyr114Cys-type TTR-related FAP. Pre- and postcontrast serial MR images at the level of the basal ganglia (A) and the cerebellum (B) are shown. T1-weighted and FLAIR images are presented in the upper and lower rows, respectively.

A, On T1-weighted MR images obtained immediately after contrast administration, leptomeningeal enhancement is equivocal because it is similar to enhanced cortical vessels. Marked enhancement of the leptomeninges is obvious on FLAIR images obtained at the same time points. At 3 hours postcontrast, there is marked CSF enhancement on both sequences. The contrast between the CSF and the brain parenchyma appears greater on FLAIR than T1-weighted images. At 6 hours postcontrast, the FLAIR image shows marked—and the T1-weighted image mild—CSF enhancement. CSF enhancement has almost disappeared on the 24-hour T1-weighted image. Mild CSF enhancement persists in some sulci on the 24-hour FLAIR image; some motion artifacts are present. There is no apparent enhancement of the intraventricular CSF or the brain parenchyma on either sequence at any time point examined.

B, On the precontrast FLAIR image, slightly hyperintense areas are seen in the right vitreous body (arrow). On images obtained immediately after contrast administration, there is mild enhancement of the leptomeninges of the left cerebellar hemisphere (arrowhead) and right auditory canal (arrow). There is no apparent leptomeningeal enhancement on T1-weighted images. On 3-hour FLAIR images, there is marked CSF enhancement in the prepontine and cerebellopontine angle cisterns and auditory canal (arrowhead). Mild enhancement of the right labyrinth (arrow) and bilateral vitreous bodies is observed. On the 3-hour, T1-weighted image, there is mild enhancement only in the prepontine and cerebellopontine angle cisterns. The 6-hour FLAIR image shows marked CSF enhancement in the prepontine and cerebellopontine angle cisterns and marked enhancement of the bilateral labyrinths (arrows). Marked and mild enhancement are seen in the right and left vitreous on the FLAIR images. The 6-hour, T1-weighted image demonstrates mild enhancement in the prepontine and cerebellopontine angle cisterns and auditory canals; there is equivocal enhancement in the right labyrinth (arrow). On 24-hour images, there is almost no CSF enhancement on both sequences. Mild enhancement is seen in both labyrinths (arrows) on both sequences. On the FLAIR image, marked and mild enhancement persist in the right and left vitreous bodies. On T1-weighted images obtained 24 hours after contrast administration, there is no obvious enhancement of the vitreous.