Hirayama Disease Neuroimaging Clinics

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Hirayama Disease Neuroimaging Clinics Yen-Lin Huang, MD, Chi-Jen Chen, MD Neuroimaging Clinics Volume 21, Issue 4, Pages 939-950 (November 2011) DOI: 10.1016/j.nic.2011.07.009 Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 1 Photograph of a 19-year-old man, note the wasting of first dorsal interosseous (curved arrow) and other small muscles of the hand with preservation of brachioradialis muscles (straight arrow) giving an appearance of oblique amyotrophy. (From Pradhan S. Bilaterally symmetric form of Hirayama disease. Neurology 2009;72:2083–9; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 2 Transverse sections of cervical spinal cord. Anterior-posterior shrinkage of bilateral anterior horns, most severely at C7 and C8, predominantly on the left. (From Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy. Neurology 2000;20:S92; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 3 A 21-year-old man with slowly progressive weakness and atrophy of hands and forearms. (A) Nonflexion sagittal T2-weighted MR image (3000/100/2) shows focal cord atrophy at the C5 to C7 levels. The posterior wall of the dural canal is in close contact with the spinal canal. (B) Nonflexion axial T2-weighted MR image (542/16/2) reveals atrophy at C7 level, more prominent at right anterior aspect of the cord (arrow). (C) Flexion sagittal T2-weighted MR image (3000/100/2) shows forward displacement of the posterior wall of the dural canal below C3 (short arrows), which causes marked flattening of the lower cervical cord. An epidural mass (long arrow) posterior to the shifting dura matter is noted, with small curvilinear flow void signals inside it. (D) Contrast-enhanced flexion sagittal T1-weighted MR image (530/12/2) shows strong and homogeneous enhancement of this epidural mass (arrow). (E) Contrast-enhanced nonflexion sagittal T1-weighted MR image (530/12/2), shows disappearance of the posterior dural mass and recovery of the shifting dura matter to its normal position. Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 4 (A) Sagittal fast-spin-echo T2-weighted MR image (4133/85) in a 17-year-old male control subject. (B) Sagittal fast-spine-echo T2-weighted MR image (3250/9103) in a 17-year-old male patient with slowly progressive weakness and atrophy of the left hand and forearm. Cervical curvature is measured according to the relationship of the dorsal aspect of the vertebral bodies C3 through C6 to a line drawn from the dorsocaudal aspect of the vertebral body C2 to the dorsocaudal aspect of the vertebral body C7 (black line in A, white line in B). By definition, normal lordotic cervical curvature is curvature in which no part of the dorsal aspect of the vertebral bodies C3 through C6 cross the line from C2 through C7 (A). An abnormal (straight or kyphotic) curvature is curvature in which part or all of the dorsal aspects of the vertebral bodies C3 through C6 meet or cross that line (B). (From Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy: neutral-position MR imaging findings—importance of loss of attachment. Radiology 2004;231:39–44; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 5 Transverse neutral-position T2∗-weighted MR image (433/17, with 20° flip angle) at the pedicular level. (A) Image in 20-year-old male control subject. (B) Image in an 18-year-old patient with Hirayama disease. The lamina, defined medially by point of junction of lamina and laterally by tangential line along medial aspect of pedicle (longest white line in A and B), is equally divided into three parts. Images show less than 33.3% LOA between the posterior dural sac and subjacent lamina in a and more than 33.3% LOA in b. Asymmetrically bilateral cord flattening (more severe on the right side) is also depicted in b. (From Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy: neutral-position MR imaging findings—importance of loss of attachment. Radiology 2004;231:39–44; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 6 Neutral-position MR images in a 24-year-old male patient with progressive bilateral weakness and atrophy, which is greater on the right side than on the left, for 3 years. (A) Sagittal fast-spin-echo T2-weighted image (4000/85) shows kyphotic cervical curvature, localized cord atrophy at C5 and C6 (arrowheads), and faint noncompressed intramedullary high signal intensity at C5-6 disk (arrow). (B) Transverse T2∗-weighted MR image (433/17, with 20° flip angle) at pedicular level of C5 shows symmetric cord flattening and 100% LOA between the posterior dural sac and subjacent lamina (arrows). (From Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy: neutral-position MR imaging findings—importance of loss of attachment. Radiology 2004;231:39–44; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions

Fig. 7 Neutral-position MR images in 18-year-old male patient with progressive right hand and forearm weakness and atrophy for 2 1/2 years. (A) Sagittal fast-spin-echo T2-weighted image (4000/85) shows normal cervical curvature, localized cord atrophy (arrowheads), and noncompressed intramedullary high signal intensity (arrow). (B) Transverse T2∗-weighted MR image (433/17, with 20° flip angle) shows asymmetric cord flattening (on right side) and nearly 100% LOA between the posterior dural sac and subjacent lamina. Intramedullary high signal intensity (arrowhead) is depicted in right-sided gray matter. (C) Sagittal flexion fast-spin-echo T2-weighted image (4000/85) shows anterior displacement of posterior dural sac (black arrows) and cord compression (white arrow). (From Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy: neutral-position MR imaging findings–Importance of loss of attachment. Radiology 2004;231:39–44; with permission.) Neuroimaging Clinics 2011 21, 939-950DOI: (10.1016/j.nic.2011.07.009) Copyright © 2011 Elsevier Inc. Terms and Conditions