J Korean Med Sci.  2016 Oct;31(10):1664-1667. 10.3346/jkms.2016.31.10.1664.

Hirayama Disease with Proximal Involvement

• Kim J
• Kim Y
• Kim S
• Oh K

• KMID: 2468261
• DOI: http://doi.org/10.3346/jkms.2016.31.10.1664

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.

Keyword

Electromyography; Hirayama Disease; Proximal Upper Limb; Secondary Disease Progression

MeSH Terms

Adult
Electromyography
Evoked Potentials, Somatosensory/physiology
Humans
Magnetic Resonance Imaging
Male
Muscle Weakness/*physiopathology
Muscle, Skeletal/diagnostic imaging
Spinal Muscular Atrophies of Childhood/*diagnosis
Upper Extremity/diagnostic imaging

Figure

• 

  Fig. 1 Gross atrophy view of the patient. (A) Distal oblique portion of the left arm. (B) Proximal portion of the left arm.

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