Key result
Case report reveals rare secondary Hirayama disease progression to proximal upper limb after 9 years.
Why the study?
Hirayama disease typically affects the distal upper limb, but cases with proximal involvement and secondary progression are rare and clinically significant.
Case Report (n=1)
No
Hirayama disease can rarely involve the proximal upper limb through secondary progression, highlighting the utility of accurate electromyography for diagnosis.
Proximal weakness may occur in longstanding Hirayama disease; this single case leaves open whether it represents progression or a phenotypic variant.
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.
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Kim et al. (2016) conducted a case report in Hirayama disease (n=1). Hirayama disease with proximal progression was evaluated on Clinical and electromyographic findings of proximal upper limb involvement. A 29-year-old man with Hirayama disease demonstrated rare secondary progression involving the proximal upper limb 9 years after initial diagnosis, confirmed by abnormal needle electromyography.
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