Archana Singh1, Chhabi2, Sudipta Saha3, Samiran Samanta4
Hirayama disease, also termed non-progressive juvenile spinal muscular atrophy of the distal upper limbs, is a type of cervical myelopathy related to flexion movements of the neck.
The objective of the study was to evaluate the role of Dynamic MR imaging findings in young patients with clinical suspicion of Hirayama disease.
MATERIALS AND METHODS
Thirteen patients (age range from 16 to 26 years) with clinical suspicion of Hirayama disease underwent thorough clinical evaluation and dynamic cervical MRI in the Department of Radiology at IPGME&R within a time duration of June 2014 to September 2016.
RESULTS
All the thirteen patients showed anterior shifting of posterior dural sac, cord flattening, abnormal curvature, enhancing epidural component. Nine of them showed localised cord atrophy and intramedullary T2 hyperintensities. Ten patients showed prominent flow voids.
CONCLUSION
Hirayama disease, a rare disease affecting young adults almost always in the second to third decades of life, is characterised by insidious onset and slowly progressive course followed by static phase of unilateral or asymmetric atrophy of the hand(s) and forearm(s) with sparing of the brachioradialis, characterised as oblique amyotrophy. Dynamic contrast MRI has accurate and characteristic findings which help in early diagnosis and early institution of therapy.
