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MRI of Five Patients with Mitochondrial Neurogastrointestinal Encephalomyopathy

¹ Department of Radiology, New York Presbyterian Hospital, Columbia-Presbyterian Center, 177 Fort Washington Ave., Milstein Hospital Bldg., Rm. 3-105, New York, NY 10032.
² Department of Radiology, College of Physicians and Surgeons, Columbia University in the City of New York, New York, NY 10032.
³ Department of Neurology, College of Physicians and Surgeons, Columbia University in the City of New York, 630 W 168th St., P&S 4-443, New York, NY 10032.

View larger version (26K): [in a new window]   Fig. 1. —Schematic drawing of mitochondrial thymidine metabolism. Loss of thymidine phosphorylase (TP) leads to increased levels of plasma thymidine and deoxyuridine, which is hypothesized to alter mitochondrial deoxynucleotide pools leading to mitochondrial DNA (mtDNA) abnormalities. TK2 = thymidine kinase, dNT2 = deoxynucleotidase, TMP = thymidine monophosphate, TTP = thymidine triphosphate.

View larger version (144K): [in a new window]   Fig. 2A. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 35-year-old man. Axial FLAIR image (TR/TE, 9,002/157; inversion time, 2,200 msec) shows confluent abnormally increased signal intensity in centrum semiovale (severity score, 4). Note sparing of subcortical U-fibers.

View larger version (148K): [in a new window]   Fig. 2B. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 35-year-old man. Axial FLAIR image (9,002/157; inversion time, 2,200 msec) shows abnormally increased signal intensity in midline corpus callosum (splenium) and internal capsules near thalami bilaterally with severity scores of 3 and 2, respectively.

View larger version (128K): [in a new window]   Fig. 2C. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 35-year-old man. Axial FLAIR image (9,002/157; inversion time, 2,200 msec) shows abnormally increased signal intensity in cerebellar white matter bilaterally (severity score, 2).

View larger version (159K): [in a new window]   Fig. 3A. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 39-year-old man. Axial FLAIR image (TR/TE, 10,002/161; inversion time, 2,220 msec) shows confluent abnormally increased signal intensity in centrum semiovale (severity score, 4). Note again sparing of subcortical U-fibers.

View larger version (139K): [in a new window]   Fig. 3B. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 39-year-old man. Axial FLAIR image (10,002/161; inversion time, 2,220 msec) shows minimal abnormally increased signal intensity in caudate heads (severity score, 1). Putamina, globi pallidi, and thalami show no abnormal signal on inferior slices (severity scores, 0, 0, and 0, respectively). Slightly increased signal intensity over both thalami is probably internal capsular white matter fanning into white matter of both corona radiata.

View larger version (148K): [in a new window]   Fig. 4A. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 39-year-old woman. Axial FLAIR image (TR/TE, 9,000/110; inversion time, 2,500 msec) shows abnormally increased signal intensity bilaterally in caudate heads, putamina, globi pallidi, and thalami (severity scores: 4, 3, 2, and 3, respectively). Also note abnormally increased signal intensity bilaterally in internal, external, and extreme capsules (severity scores: 3, 4, and 4, respectively). Gray matter of claustrum is suggested between external and extreme capsules.

View larger version (122K): [in a new window]   Fig. 4B. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 39-year-old woman. Axial FLAIR image (9,000/110; inversion time, 2,500 msec) shows abnormally increased signal intensity in pons (severity score, 2).

View larger version (125K): [in a new window]   Fig. 5A. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 46-year-old man. Axial FLAIR image (TR/TE, 10,002/147; inversion time, 2,200 msec) shows extensive abnormally increased signal intensity in corpus callosum (splenium), caudate heads, putamina, globi pallidi, and thalami (severity scores: 2, 3, 3, 3, and 3, respectively). Also note abnormally increased signal intensity bilaterally in internal, external, and extreme capsules (severity scores: 3, 4, and 3, respectively).

View larger version (139K): [in a new window]   Fig. 5B. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 46-year-old man. Axial FLAIR image (10,002/147; inversion time, 2,200 msec) shows abnormally increased signal intensity in midbrain (severity score, 4).

View larger version (136K): [in a new window]   Fig. 5C. —MR images of mitochondrial neurogastrointestinal encephalomyopathy in 46-year-old man. Axial FLAIR image (10,002/147; inversion time, 2,200 msec) shows abnormally increased signal intensity in cerebellar white matter and pons (severity scores: 3 and 3, respectively).

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