A Korean Case of Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease) with Dynamic Cervical Cord Compression.
10.3346/jkms.2004.19.5.768
- Author:
Ohyun KWON
1
;
Manho KIM
;
Kwang Woo LEE
Author Information
1. Department of Neurology, Seoul National University Hospital, Seoul, Korea. kimmanho@snu.ac.kr
- Publication Type:Case Report ; Research Support, Non-U.S. Gov't
- Keywords:
Magnetic Resonance Imaging;
Motor Neuron Disease;
Spinal Cord Compression
- MeSH:
Adolescent;
Cervical Vertebrae;
Humans;
*Magnetic Resonance Imaging;
Male;
Motor Neuron Disease/complications/pathology;
Research Support, Non-U.S. Gov't;
Spinal Cord Compression/*etiology/*pathology;
Spinal Muscular Atrophies of Childhood/*complications/*pathology
- From:Journal of Korean Medical Science
2004;19(5):768-771
- CountryRepublic of Korea
- Language:English
-
Abstract:
We present a Korean case of Hirayama disease with its typical neuroradiological findings of forward displacement of cervical dural sac and compression of the lower cervical cord during neck flexion. A 15-yr-old boy was presented with a one-year history of progressive weakness and atrophy affecting bilateral hands and forearms. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. Presumably, this disease might have been prevalent in Korea frequently under the diagnosis of "benign focal amyotrophy". In this regard, we discuss the clinical importance of cervical MRI with neck flexion and anticipate the increasing reports of the case substantiated by its characteristic radiological features.
