Abstract
A 21-year-old man with a muscular atrophy of the left distal upper extremity is presented. The disorder had been progressive over a few years, showing an exacerbation of the hand's weakness when the patient worked in a chilled environment (i.e., in a cold room). The patient's diagnostic work-up was extensive and the MRI documented the presence of a cervical myelopathy, associated to an inversion of the physiological lordosis at the C5-C6 level, with a phenotype highly resembling Hirayama disease. This case indirectly supports the debated hypothesis that juvenile amyotrophy of the upper limb (Hirayama disease) is actually a type of cervical myelopathy, with a likely ischaemic pathogenesis of the ventral horns.
MeSH terms
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Arm / innervation
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Arm / pathology
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Arm / physiopathology
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Cervical Vertebrae / pathology*
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Cervical Vertebrae / physiopathology
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Cold Temperature / adverse effects
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Cumulative Trauma Disorders / complications
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Cumulative Trauma Disorders / pathology*
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Cumulative Trauma Disorders / physiopathology
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Disease Progression
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Functional Laterality / physiology
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Humans
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Ischemia / etiology
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Ischemia / pathology
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Ischemia / physiopathology
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Kyphosis / complications
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Kyphosis / pathology*
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Kyphosis / physiopathology
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Magnetic Resonance Imaging
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Male
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Muscle Weakness / etiology
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Muscle Weakness / pathology
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Muscle Weakness / physiopathology
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Muscle, Skeletal / innervation
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Muscle, Skeletal / pathology
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Muscle, Skeletal / physiopathology
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Muscular Atrophy, Spinal / etiology
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Muscular Atrophy, Spinal / pathology*
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Muscular Atrophy, Spinal / physiopathology
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Neural Pathways / injuries
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Neural Pathways / pathology
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Neural Pathways / physiopathology
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Occupational Diseases / complications
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Occupational Diseases / pathology*
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Occupational Diseases / physiopathology
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Spinal Cord / blood supply
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Spinal Cord / pathology
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Spinal Cord / physiopathology
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Spinal Cord Compression / etiology
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Spinal Cord Compression / pathology*
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Spinal Cord Compression / physiopathology
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Syndrome
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Young Adult