Hirayama disease

Case contributed by A.Prof Frank Gaillard

Presentation

Bilateral hand and forearm muscle wasting consistent with denervation.

Patient Data

Age: 30 years
Gender: Male
Modality: MRI

Multiplanar, multisequence imaging has been obtained through the cervical spine, including T2 sagittal and axial views during flexion. 

Vertebral height, alignment (other than straightening pole of the cervical spine thought to be positional) and bone marrow signal unremarkable.

The cord is markedly thinned and demonstrates high T2 signal between C5/6 and C6/7 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\XB0\ of flexion was achieved (foramen magnum to T4). The posterior wall of the dural canal displaces anteriorly from C5 to T2, the epidural space filling with intermediate to high T2 signal material (thought to represent 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 characteristic appearances of Hirayama disease, and elegantly demonstrates the need for flexion views to confirm the diagnosis. 

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Case Information

rID: 19380
Case created: 2nd Sep 2012
Last edited: 17th Jan 2016
Inclusion in quiz mode: Included

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