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The Korean Journal of Pathology 1981;15(3): 300-304.
척수병인성 관절만곡증과 동반된 뇌의 이소성 회백질 -1예보고-
Heteropic Gray Matter of the Brain Associated with Myelopathic Arthrogryposis -An autopsy case report-
ABSTRACT
The heterotopia of cerebral gray matter is a rare malformation, which could serve as epileptogenic focus in some patients. This anomaly of neuronal migration, however, can occur as a minor isolated phemomenon. Authors observed gary matter heterotopia in frontal white matter, that was incidentally found at an autopsy done in a 7-year old toy who died after corrective surgery for the congenital multiple flexion deformities. The cerebral heterotopia was found near the lateral corner of the frontal horn of the left lateral ventricle, measuring 1 cm. in maximum extent, and was located under the abnormally deep sulcus of the left superior frontal gyrus. Several separate satellite gray nodules were seen in this area. There was another lesion located slightly caudal to the first one at the same side and was composed of a round mass of 5mm size above the head of the caudate nucleus. Microscopically the heterotopic nodules consisted of relatively well formed nerve cells of cortical type. However, their orientation and cellularity were not those of normal cortex. Additionally a marked decrease in the number of anterior horn cells in the spinal cord in the cervical and lumbar levels were seen, indicative of myelopathic nature of arthrogryposis, together with skeletal muscle findings.