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- W2169550427 abstract "Hirayama disease, also known as monomelic amyotrophy, is a relatively benign motor neuron disease, characterized by insidious muscle wasting and weakness, unilateral or asymmetric restricted to the distal upper limb, followed by a quiescent phase after onset in the second to third decades.1 Pathophysiology is uncertain and no treatment is available to restore weakness.2 Although the degree of disability does not interfere with activities of daily living,2,3 the appearance of the hand is of major concern to the patient but is often minimized by clinicians concerned about the cause and the weakness. Here, we describe hand rejuvenation surgery, a procedure that restores youthful appearance to the hand, as a treatment of a patient's chief complaint in Hirayama disease.Level of evidence is class IV: single observational study without controls.### Case report.Hirayama disease was diagnosed in a 36-year-old man with chief complaints of left hand atrophy and weakness which was noted soon after age 13 and did not progress after age 20. On examination, marked atrophy of left intrinsic hand muscles and forearm flexors was noted. Slight weakness was demonstrated in the C7, C8, and T1 myotomes. EMG disclosed a slightly active and chronic neurogenic pattern in the appropriate spinal segments. MRI revealed asymmetric anterior flattening and atrophy …" @default.
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- W2169550427 date "2011-06-20" @default.
- W2169550427 modified "2023-09-26" @default.
- W2169550427 title "Treating the chief complaint: Hand rejuvenation for Hirayama disease" @default.
- W2169550427 cites W1980414304 @default.
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- W2169550427 doi "https://doi.org/10.1212/wnl.0b013e3182242da7" @default.
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