title: Hirayama Disease with Proximal Involvement.
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.
authors.affiliation: Department of Physical Medicine and Rehabilitation, Soonchunhyang University Cheonan Hospital, Cheonan, Korea.
authors.forename: Jinil
authors.identifier: http://orcid.org/0000-0002-4706-7259
authors.initials: J
authors.lastname: Kim
authors.affiliation: Department of Physical Medicine and Rehabilitation, Soonchunhyang University Cheonan Hospital, Cheonan, Korea.
authors.forename: Yuntae
authors.identifier: http://orcid.org/0000-0003-4063-4692
authors.initials: Y
authors.lastname: Kim
authors.affiliation: Department of Physical Medicine and Rehabilitation, Soonchunhyang University Cheonan Hospital, Cheonan, Korea.
authors.forename: Sooa
authors.identifier: http://orcid.org/0000-0003-1578-0452
authors.initials: S
authors.lastname: Kim
authors.affiliation: Department of Physical Medicine and Rehabilitation, Soonchunhyang University Cheonan Hospital, Cheonan, Korea. okokykkh@schmc.ac.kr.
authors.forename: Kiyoung
authors.identifier: http://orcid.org/0000-0002-1886-5462
authors.initials: K
authors.lastname: Oh
mesh_terms: D000328:Adult; D004576:Electromyography; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis*; D034941:Upper Extremity / Q000000981:diagnostic imaging
keywords: Electromyography; Hirayama Disease; Proximal Upper Limb; Secondary Disease Progression