脊髓脊病因性 關節彎齒症과 동반된 腦의 異所性 灰鹵嶺 -1例報告-
Heteropic Gray Matter of the Brain Associated with Myelopathic Arthrogryposis -An autopsy case report-
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서정일/Jung Il Suh
박문향/바효숙/김기용/지제근/Moon Hyang Park/Hyo Sook Park/Key Tong Kim/Je G. Chi
KMID : 0357919810150030300
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.
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