Presentation
Bilateral hand and forearm muscle wasting consistent with denervation.
Patient Data
The cord is markedly thinned and demonstrates high T2 signal between C5 to C7 and consistent with myelomalacia. In neutral position the canal is capacious, and no cord compression is present. The neural exit foramina at all levels are capacious.
The patient was then asked to flex the cervical spine, and the head was kept in this position with foam wedges while sagittal and axial T2 acquisitions were repeated. Approximately 40 degrees of flexion was achieved (foramen magnum to T4). The posterior theca displaces anteriorly from C5 to T2, the epidural space filling with engorged epidural venous plexus.
The cord appears bow-strung over the posterior aspect of the vertebral bodies and compressed by the aforementioned displaced dura.
Case Discussion
This case illustrates the characteristic appearances of Hirayama disease, and elegantly demonstrates the need for flexion views to confirm the diagnosis.
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