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Case Discussion-Neuroradiology

This is 22 year old male with weakness of the both hands and on clinical examination it was C8/T1 distribution. MRI done outside was reported as unremarkable except for early discovertebral changes. Referred to us for a repeat study, we noticed cord atrophy and signal alteration at C4-C6 levels, which couldn’t be explained with disc disease. There was no history of trauma. No AVM was evident. Vascular cause was suspected. Hirayama disease is one of the differentials in such an appearance and it is mandatory to do a flexion study in cases like this. On flexion the diagnosis was clinched by anterior movement of posterior dura and engorged epidural veins seen as prominent flow voids. Your views are welcome. We encourage readers to discuss their doubts.

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Discussion-Hirayama Disease-On myelograms and flexion-extension MR images, forward migration of the posterior wall of the dura mater is observed. The posterior epidural space becomes enlarged with flexion and is seen as a crescent of high signal intensity on T1-
and T2-weighted MR images, with or without epidural flow voids. Compressive flattening of the spinal cord accompanies the forward shifting of the posterior dura. The changes are often greatest at the C6 vertebral level . In the majority of cases, spinal cord flattening is asymmetric. In later stages of the disease, spinal cord atrophy ensues and is generally limited to the anterior horn cells region. Morphologic changes on MR images correlate with clinical and electromyographic data
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Case Discussion-Neuroradiology Reviewed by Sumer Sethi on Wednesday, January 21, 2009 Rating: 5


Unknown said...

when will we say a cord to be atrophic? is there any definite limit?

Sumer Sethi said...

visibly if you look at the cervical in the sagittal neutral view you will seen it is showing some atrophy, hirayam disease is a specific diagnosis and i have diagnosed along with my neurologist team three such cases in last 2 years..

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