Hirayama Disease with Proximal Involvement.
10.3346/jkms.2016.31.10.1664
- Author:
Jinil KIM
1
;
Yuntae KIM
;
Sooa KIM
;
Kiyoung OH
Author Information
1. Department of Physical Medicine and Rehabilitation, Soonchunhyang University Cheonan Hospital, Cheonan, Korea. okokykkh@schmc.ac.kr
- Publication Type:Case Report
- Keywords:
Electromyography;
Hirayama Disease;
Proximal Upper Limb;
Secondary Disease Progression
- MeSH:
Adult;
Atrophy;
Diagnosis;
Electromyography;
Evoked Potentials, Somatosensory;
Humans;
Magnetic Resonance Imaging;
Motor Neuron Disease;
Muscle Weakness;
Muscles;
Needles;
Upper Extremity
- From:Journal of Korean Medical Science
2016;31(10):1664-1667
- CountryRepublic of Korea
- Language:English
-
Abstract:
Hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. A 29-year-old man visited the hospital with a 1-year history of weakened left proximal upper limb. He was diagnosed with Hirayama disease 9 years ago, while there was no further progression of the muscle weakness afterward. Atrophy and weakness was detected in proximal upper limb muscles. Magnetic resonance imaging and somatosensory evoked potentials were normal. Needle electromyography showed abnormal findings in proximal upper limb muscles. Our patient had Hirayama disease involving the proximal portion through secondary progression. Clinical manifestation and accurate electromyography may be useful for diagnosis. Rare cases with progression patterns as described here are helpful and have clinical meaning for clinicians.
