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Fig 3. Course of cerebellar MR imaging findings in patient 8. Left column, T1-weighted images (T1WI); right column, T2-weighted images (T2WI). The first signal intensity abnormalities in the cerebellum were detected at the age of 4 years (1994) and were composed of subtle increased signal intensities on T2WI limited to the region of the dentate nucleus. In 1997, the signal intensity abnormalities on T1- and T2WI involved the dentate nucleus, surrounding white matter, middle cerebellar peduncle, and pons. In 2001, the patient developed an obstructive hydrocephalus, a thinning of the corpus callosum, and progressive cerebellar neurologic symptoms. At the last follow-up (2005), the dentate nucleus appeared atrophic.