Juvenile muscular atrophy of distal upper extremity (Hirayama disease)
Abstract
This disease is characterized by initially progressive muscular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a spontaneous arrest within several years. This disease has been thought to be separate from motor neuron diseases, yet some authors still consider the illness a variant of motor neuron disease. However, the pathological evidence of ischemic changes in the lower cervical anterior horn should facilitate differentiation of the disorder from degenerative motor neuron disease. Recent radiological investigations proved compressive flattening of the lower cervical cord due to forward displacement of the cervical dural sac and spinal cord induced by neck flexion. These findings suggest that sustained or repeated neck flexion may cause ischemic changes in the cervical anterior horn. Application of a cervical collar to minimize neck flexion prevents progressive muscular weakness in an early stage of the disease.
Read the paper
Related studies
- The potential and limitations of neutrons, electrons and X-rays for atomic resolution microscopy of unstained biological molecules
- X-ray-Computed Tomography Contrast Agents
- Reactivity of Surface Species in Heterogeneous Catalysts Probed by In Situ X-ray Absorption Techniques
- Single-crystal X-ray diffraction studies on structural transformations of porous coordination polymers
- Investigation of solid/vapor interfaces using ambient pressure X-ray photoelectron spectroscopy
- Resonant X-Ray Scattering Studies of Charge Order in Cuprates
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.