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Case Reports
. 2020 Jul-Aug;34(4):2153-2157.
doi: 10.21873/invivo.12022.

Vietnamese Case Series of Hirayama Disease

Nguyen Duy Hung #  1   2 , Nguyen Minh Duc #  3   4 , Nguyen Thanh VAN  2 , LE Thanh Dung  2 , Hoang Duc Ha  5 , Nguyen Duy Hue  1   2
Affiliations
Case Reports

Vietnamese Case Series of Hirayama Disease

Nguyen Duy Hung et al. In Vivo. 2020 Jul-Aug.

Abstract

Background/aim: Hirayama disease, a rare neurological disorder, is characterized by progressive, asymmetric muscle atrophy of the forearm and hand in young male patients.

Case report: We noted two cases of Hirayama disease that appeared in the right upper limb and identified the cervical neutral and flexion position magnetic resonance imaging (MRI) characteristics.

Conclusion: In the flexion posture, the cervical MRI allows early diagnosis of Hirayama disease and should be considered in patients with the typical clinical symptoms.

Keywords: Hirayama disease; cervical MRI; flexion position; neutral position.

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Conflict of interest statement

There are no conflicts of interest to declare regarding this study.

Figures

Figure 1
Figure 1. A 16-year-old male clinically suggested to have Hirayama disease. (A) and (B) The atrophy of dorsal interosseous muscles of the hand, the thenar, hypothenar muscles. (C) The atrophy of the medial aspect of forearm muscles with preservation of the brachioradialis
Figure 2
Figure 2. Cervical MRI of the 16-year-old patient in the neutral position. (A) Sagittal T2-weighted image showing a focal atrophy of the spinal cord at C5-C6 levels with no signal abnormality (arrow) (B) Axial T2-weighted image at C6 level showing flattening of the cord (arrow)
Figure 3
Figure 3. Cervical MRI of the 16-year-old patient in the flexion position. (A) Sagittal T2-weighted and (B) Axial T2-weighted images at C5 level showing the posterior dura was displaced anteriorly (arrow) causing the widening of posterior epidural space (asterisk) and spinal stenosis. The spinal cord at C5-C6 levels was compressed between the dura posteriorly and the vertebrae anteriorly.
Figure 4
Figure 4. An 18-year-old male clinically suggested to have Hirayama disease. (A) and (B) The wasting of the dorsal interosseous muscle and the palmar aspect muscles, as well as weakness in finger extension. (C) The wasting of the medial aspect of forearm muscles with preservation of the brachioradialis
Figure 5
Figure 5. Cervical MRI of the 18-year-old patient in the neutral position. (A) Sagittal T2-weighted image showing a subtle medullary atrophy at C5-C6 levels with no signal abnormality (arrow) (B) Axial T2-weighted image at C6 level showing a flattening of the cord and detachment of the posterior dura (arrow)
Figure 6
Figure 6. Cervical MRI of the 18-year-old patient in the flexion position. (A) Sagittal T2-weighted and (B) Axial T2-weighted images at C6 level showing the enlargement of the posterior epidural space due to the anterior displacement of the posterior dura causing the spinal stenosis
See this image and copyright information in PMC

References

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