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. 2010 Nov;20(4):245-9.
doi: 10.4103/0971-3026.73528.

MRI findings in Hirayama disease

Monali Raval  1 , Rima Kumari, Aldrin Anthony Dung Dung, Bhuvnesh Guglani, Nitij Gupta, Rohit Gupta
Affiliations

MRI findings in Hirayama disease

Monali Raval et al. Indian J Radiol Imaging. 2010 Nov.

Abstract

The objective of the study was to study the magnetic resonance imaging (MRI) features of Hirayama disease on a 3 Tesla MRI scanner. Nine patients with clinically suspected Hirayama disease were evaluated with neutral position, flexion, contrast-enhanced MRI and fast imaging employing steady-state acquisition (FIESTA) sequences. The spectrum of MRI features was evaluated and correlated with the clinical and electromyography findings. MRI findings of localized lower cervical cord atrophy (C5-C7), abnormal curvature, asymmetric cord flattening, loss of attachment of the dorsal dural sac and subjacent laminae in the neutral position, anterior displacement of the dorsal dura on flexion and a prominent epidural space were revealed in all patients on conventional MRI as well as with the dynamic 3D-FIESTA sequence. Intramedullary hyperintensity was seen in four patients on conventional MRI and on the 3D-FIESTA sequence. Flow voids were seen in four patients on conventional MRI sequences and in all patients with the 3D-FIESTA sequence. Contrast enhancement of the epidural component was noted in all the five patients with thoracic extensions. The time taken for conventional and contrast-enhanced MRI was about 30-40 min, while that for the 3D-FIESTA sequence was 6 min. Neutral and flexion position MRI and the 3D-FIESTA sequence compliment each other in displaying the spectrum of findings in Hirayama disease. A flexion study should form an essential part of the screening protocol in patients with suspected Hirayama disease. Newer sequences such as the 3D-FIESTA may help in reducing imaging time and obviating the need for contrast.

Keywords: 3D-FIESTA sequence; Flexion MR; Hirayama disease.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1 (A, B)
Figure 1 (A, B)
Neutral position T2W sagittal (A) and T2W axial (B) magnetic resonance images show localized cervical cord atrophy at the C5-C7 levels (arrows), which is asymmetrical on the axial image (arrow) with a pear-shaped cross-sectional appearance, with straightening of the cervical curvature and loss of attachment of the dorsal dura (arrowhead)
Figure 2
Figure 2
T2W sagittal magnetic resonance image in flexion shows anterior displacement of the dorsal dura (arrow) compressing the thecal sac, with a prominent dorsal epidural compartment (arrowhead)
Figure 3 (A, B)
Figure 3 (A, B)
Sagittal flexion (A) and axial (B) contrast-enhanced magnetic resonance images show an enhancing epidural crescent (arrows)
Figure 4 (A, B)
Figure 4 (A, B)
Sagittal flexion (A) and axial (B) 3D fast imaging employing steady-state acquisition magnetic resonance images show prominent flow voids within the dorsal epidural space (arrows) with asymmetric cord compression
See this image and copyright information in PMC

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