AI Article Synopsis
- The autopsy detailed the case of a 67-year-old man diagnosed with spinal muscular atrophy (SMA) type III, characterized by slowly progressive muscle weakness and wasting in the extremities, similar to that of his brother's condition.
- Examination of the spinal cord revealed significant neuronal loss and gliosis in the anterior horns, atrophic spinal roots, and neurogenic changes in skeletal muscles, indicating severe degeneration.
- Pathological findings were similar to those seen in SMA type I, with prominent chronic changes and mild acute changes, reflecting the long duration of the man's clinical symptoms.
Article Abstract
We report an autopsy case of a 67-year-old man clinicogenetically diagnosed as having spinal muscular atrophy (SMA) type III (Kugelberg-Welander disease), showing slowly progressive muscle wasting and weakness of the extremities. His brother showed similar manifestations. Autopsy revealed neuronal loss and severe gliosis in the anterior horns of the spinal cord, a marked neurogenic change of skeletal muscles and mild degeneration of cardiomyocytes. Chromatolytic change was seen in the anterior horn, but not in the Clarke's and thalamic nuclei. The anterior spinal roots were atrophic, and there was loss of myelinated fibers with abundant glial bundles. In addition, degeneration was also observed in the posterior column and dentate nucleus. The pathological features were essentially similar to those of SMA I. Chronic change was prominent while acute change was mild in degree, corresponding to a very long clinical course.
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| http://dx.doi.org/10.1111/j.1440-1789.2008.00910.x | DOI Listing |
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