Article Figures & Data
Figures
- 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).
- 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.
- 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.
Tables
Atrophy, abnormal signals, and FA of patients with MERRF and healthy controls
Atrophy Abnormal Signals FA (z score) SCP MCP Cerebellum Brain Stem Cerebrum Brain Stem Basal Ganglia SCP MCP Patient 1 ++ + + + – + – 0.469 (−0.7) 0.473 (−1.3) 2 + ± + + + + + 0.550 (0.6) 0.465 (−1.7) 3 + – + – + – – 0.513 (0.0) 0.494 (−0.4) Healthy controls – – – – – – – 0.513 ± 0.066 0.503 ± 0.023 Note:—SCP indicates superior cerebellar peduncles; MCP, middle cerebellar peduncles; ++, severely present; +, mildly present; −, absent; ±, equivocal.
