AJDRAJNR - American Journal of Neuroradiology

Published ahead of print on November 7, 2007
doi: 10.3174/ajnr.A0865
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Clinical and Brain MR Imaging Features Focusing on the Brain Stem and Cerebellum in Patients with Myoclonic Epilepsy with Ragged-Red Fibers due to Mitochondrial A8344G Mutation

S. Itoa, W. Shiraia, M. Asahinaa and T. Hattoria

a From the Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan


Figure 1
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  		Fig 1. T2-weighted axial images in patient 1 at 22 years of age (disease duration, 14 years). A, The mid pons and midbrain are slightly atrophic, and the middle and superior cerebellar peduncles show mild atrophy. B, The periaqueductal gray matter signal intensity is abnormally hyperintense (arrows).


Figure 2
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  		Fig 2. T2-weighted axial images in patient 1 at 24 years of age (disease duration, 16 years). A, There is atrophy of the mid pons, middle cerebellar peduncles, and cerebellar hemisphere and severe atrophy of the superior cerebellar peduncles. B, Marked abnormal hyperintensities are found bilaterally around the periaqueductal gray matter (arrows), and the midbrain tegmentum shows atrophy.


Figure 3
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  		Fig 3. T2-weighted axial images in patient 2 at 52 years of age (disease duration, 17 years). A, There is moderate atrophy of the midbrain and mild atrophy of the pons, middle cerebellar peduncles, cerebellum, and cerebrum. The superior cerebellar peduncles are also atrophic. B, There are symmetric T2 hyperintensities in the midbrain tegmentum around the periaqueductal gray matter.





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