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Related Experiment Video

Updated: Mar 24, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Imaging in Hirayama disease.

Keshav Gupta¹, Shashank Sood¹, Jayesh Modi¹

¹Department of Radiology, Medanta, The Medicity Hospital, Gurgaon, Haryana, India.

Journal of Neurosciences in Rural Practice

|March 3, 2016

Summary

Hirayama disease, a rare spinal muscular atrophy, affects young males with distal upper limb atrophy. MRI reveals characteristic cervical cord changes, especially during neck flexion, aiding diagnosis.

Area of Science:

Neurology
Radiology
Genetics

Background:

Hirayama disease, or Sobue disease, is a rare, nonprogressive spinal muscular atrophy.
It primarily affects young males, presenting with distal upper limb atrophy.

Observation:

This case series details three young males diagnosed with Hirayama disease.
Clinical presentation included atrophy of the distal upper limb.
Diagnostic imaging, particularly MRI, was crucial for identifying characteristic changes.

Findings:

Neutral position MRI showed loss of cervical lordosis and lower cervical cord atrophy.
Flexion MRI revealed anterior displacement of the posterior dura, compressing the thecal sac.
Widened posterior epidural space and flow voids were evident on 3D-CISS MRI sequences.

Keywords:

Cervical Hirayama dura

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Implications:

These findings enhance understanding of Hirayama disease's clinico-radiological features.
MRI findings during flexion are key diagnostic indicators.
Conservative management was employed for all patients in this series.