PMC4185148_01
Axial CT and Sagittal MRI of the brain show significant age-inappropriate diffuse cerebral atrophy.
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Magnetic resonance imaging (MRI) with fluid attenuated inversion recovery (FLAIR), in addition to age-inappropriate atrophy already demonstrated on CT, showed a few scattered foci of increased T2 and FLAIR (Fig. 1) signal in the subcortical white matter bilaterally which are non-specific.
CT of the abdomen with oral contrast shows marked hepatic steatosis and ascites.
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Abdominopelvic CT without IV contrast (Fig. 2) was obtained, which demonstrated marked ascites with anasarca, and a small fatty liver.
Pathologic examination of bone marrow demonstrating focal gelatinous substance (arrows), consistent with 'starvation' bone marrow.
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Bone marrow histopathology and flow cytometry revealed infiltration by gelatinous substance, fat atrophy and marrow hypocellularity (Fig. 3), with normal female karyotype and no evidence of occult malignancy.