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Monomelic Juvenile Spinal Muscular Atrophy of the Arm
Authors:Yamamoto Kiyoshi  Takase Yoshitaka  Morimatsu Mitsunori
Abstract:In 3 patients with monomelic juvenile spinal muscular atrophy, T2-weighted and gradient-echo sagittal magnetic resonance images (MRIs) demonstrated cord atrophy at the C6-7 level. T1-weighted and gradient-echo axial images revealed cord flattening at the C6-7 level. Gradient-echo axial images showed high intensity from the anterior horns to the lateral columns bilaterally at C5-7 in 1 patient and in the right anterior horns at C5-7 in 2. With the neck flexed, T1-weighted and gradient-echo sagittal images of all patients showed narrowing and anterior shifting of the dural sac, corresponding to the localized atrophy, and also congestion of the internal vertebral venous plexus. These findings correlated to clinical features and with necropsy results. The distribution of spinal cord lesions approximates the watershed areas between the central and the peripheral arteries.
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