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Case 68: Hirayama Disease¹

¹ From the Departments of Diagnostic Imaging (D.G., M.G.) and Neurology (P.R.B.), Ottawa Hospital, Civic Campus, 1053 Carling Ave, Ottawa, ON, Canada K1Y 4E9; and Division of Neuroradiology, Department of Radiology, University of Michigan Hospitals, Ann Arbor (R.J.). Received August 30, 2002; revision requested October 31; revision received November 19; accepted January 15, 2003. Address correspondence to M.G. (e-mail: mgoyal@ottawahospital.on.ca).

View larger version (101K): [in a new window]   Figure 1a. (a) Midline sagittal T1-weighted (repetition time msec/echo time msec, 500/15) MR image of the cervical spine in neutral position reveals focal spinal cord atrophy (arrow) at C5 through C6. (b) Midline sagittal T2-weighted (2,800/90) MR image of the cervical spine in neutral position shows a small area of high signal intensity (arrow), which is possibly indicative of gliosis, in the atrophied segment of the spinal cord.

View larger version (96K): [in a new window]   Figure 1b. (a) Midline sagittal T1-weighted (repetition time msec/echo time msec, 500/15) MR image of the cervical spine in neutral position reveals focal spinal cord atrophy (arrow) at C5 through C6. (b) Midline sagittal T2-weighted (2,800/90) MR image of the cervical spine in neutral position shows a small area of high signal intensity (arrow), which is possibly indicative of gliosis, in the atrophied segment of the spinal cord.

View larger version (112K): [in a new window]   Figure 2a. T1-weighted (500/15) sagittal MR images of the cervical spine in (a) extension and (b) flexion. Note the spacious cervical dural canal and presence of wide subarachnoid space ventral to the spinal cord at C5-6 (arrow in a). The posterior dura (arrowhead) has shifted ventrally with flexion of the cervical spine. The epidural plexus is larger and contains multiple flow voids (arrow in b). (c) Contrast-enhanced T1-weighted (500/15) MR image of the cervical spine in flexion shows enhancement of the large posterior epidural plexus (arrows).

View larger version (103K): [in a new window]   Figure 2b. T1-weighted (500/15) sagittal MR images of the cervical spine in (a) extension and (b) flexion. Note the spacious cervical dural canal and presence of wide subarachnoid space ventral to the spinal cord at C5-6 (arrow in a). The posterior dura (arrowhead) has shifted ventrally with flexion of the cervical spine. The epidural plexus is larger and contains multiple flow voids (arrow in b). (c) Contrast-enhanced T1-weighted (500/15) MR image of the cervical spine in flexion shows enhancement of the large posterior epidural plexus (arrows).

View larger version (112K): [in a new window]   Figure 2c. T1-weighted (500/15) sagittal MR images of the cervical spine in (a) extension and (b) flexion. Note the spacious cervical dural canal and presence of wide subarachnoid space ventral to the spinal cord at C5-6 (arrow in a). The posterior dura (arrowhead) has shifted ventrally with flexion of the cervical spine. The epidural plexus is larger and contains multiple flow voids (arrow in b). (c) Contrast-enhanced T1-weighted (500/15) MR image of the cervical spine in flexion shows enhancement of the large posterior epidural plexus (arrows).

View larger version (138K): [in a new window]   Figure 3a. Transverse CT scans of the cervical spine obtained after myelography at C5-6 in (a) extension and (b) flexion. The spinal cord is atrophied with disproportionate involvement of the right hemicord (arrow in a). Anterior subarachnoid space is completely effaced, the spinal cord is compressed, and posterior dura is shifted ventrally (arrows in b).

View larger version (120K): [in a new window]   Figure 3b. Transverse CT scans of the cervical spine obtained after myelography at C5-6 in (a) extension and (b) flexion. The spinal cord is atrophied with disproportionate involvement of the right hemicord (arrow in a). Anterior subarachnoid space is completely effaced, the spinal cord is compressed, and posterior dura is shifted ventrally (arrows in b).