We hypothesized that previously reported cerebral atrophy may be related to chronic hypoxia in the brain (12), because defects in ZPR1 in critically ill SMA patients contribute to phrenic nerve axonal loss of function and myelin proliferation, leading to defects in diaphragmatic respiratory function causing respiratory muscle weakness, which ultimately can lead to chronic hypoxia in the brain (10), Hypoxia can cause brain MRI signal changes, and abnormal signals may appear in the basal ganglia, thalamus and surrounding cortex (13). The gene discussed is ZPR1; the disease is proximal spinal muscular atrophy.