• Journal of Korean Neurosurgical Society
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• 제55권5호
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• pp.244-247
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• 2014

Objective : The aim of this study was to evaluate the microanatomy and histological features of the central myelin in the root exit zone of facial nerve. Methods : Forty facial nerves with brain stem were obtained from 20 formalin fixed cadavers. Among them 17 facial nerves were ruined during preparation and 23 root entry zone (REZ) of facial nerves could be examined. The length of medial REZ, from detach point of facial nerve at the brain stem to transitional area, and the thickness of glial membrane of central myelin was measured. We cut brain stem along the facial nerve and made a tissue block of facial nerve REZ. Each tissue block was embedded with paraffin and serially sectioned. Slices were stained with hematoxylin and eosin (H&E), periodic acid-Schiff, and glial fibrillary acid protein. Microscopy was used to measure the extent of central myelin and thickness of outer glial membrane of central myelin. Thickness of glial membrane was examined at two different points, the thickest area of proximal and distal REZ. Results : Special stain with PAS and GFAP could be differentiated the central and peripheral myelin of facial nerve. The length of medial REZ was mean 2.6 mm (1.6-3.5 mm). The glial limiting membrane of brain stem is continued to the end of central myelin. We called it glial sheath of REZ. The thickness of glial sheath was mean $66.5{\mu}m(40-110{\mu}m$) at proximal REZ and $7.4{\mu}m(5-10{\mu}m$) at distal REZ. Conclusion : Medial REZ of facial nerve is mean 2.6 mm in length and covered by glial sheath continued from glial limiting membrane of brain stem. Glial sheath of central myelin tends to become thin toward transitional zone.

• Journal of Genetic Medicine
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• 제10권2호
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• pp.88-93
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• 2013

Alexander disease (ALXD) is a rare demyelinating disease of the white matter of the brain that is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. The overexpression of GFAP in astrocytes induces a failure in the developmental growth of the myelin sheath. The neurodegenerative destruction of the myelin sheath of the white matter is accompanied by an accumulation of abnormal deposits of Rosenthal fibers in astrocytes, which is the hallmark of ALXD. The disease can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. Early-onset disease is more severe, progresses rapidly, and results in a shorter life span than late-onset cases. Magnetic resonance imaging and genetic tests are mostly used for diagnostic purposes. Pathological tests of brain tissue for Rosenthal fibers are definitive diagnostic methods. Therapeutic strategies are being investigated. Ceftriaxone, which is an enhancer of glial glutamate transporter (GLT-1) expression, is currently in clinical trials for the treatment of patients with ALXD. To date, there are no clinically available treatments. The cause, pathology, pathophysiology, inheritance, clinical features, diagnosis, and treatment of ALXD will be reviewed comprehensively.

• 대한수의학회지
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• 제33권2호
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• pp.295-300
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• 1993

홍역이환개의 중추신경계에서 발생한 수초탈락을 수반하는 뇌척수염을 병리조직학적으로 관찰하고 조직반응과 수축탈락의 과정을 알아보기 위하여 중추신경계 조직을 파라핀 포매, 절편을 만들고 병소가 빈발하는 소뇌와 시신경로는 수초의 주요 구성단백인 MBP와 MAG 그리고 별아교세포의 Marker인 GFAP의 항혈청을 반응시켜 면역세포학적으로 관찰하였다. 조직학적으로는 대뇌에서 신경세포의 일부괴사, 신경아교세포의 결절형성, 소뇌에서는 4뇌실에 인접하여 백질부에서 수초의 공포변성, 수초탈락과 염증세포의 침윤이 현저하였고 피질부의 과립층에 인접한 부위와 시신경로에서도 수초탈락이 인정되었다. MBP와 MAG를 면역반응시킨 결과 수초의 공포화와 수초탈락이 현저한 부위에서는 MBP와 MAG가 동시에 소실되었고 부위에 따라서는 MBP또는 MAG가 먼저 소실되기도 하였다. 동시에 별아교세포의 반응은 수초탈락 초기에는 GFAP양성의 섬유와 세포가 크게 증가한 반면 수초탈락이 심하게 진행된 부위에서는 오히려 소실되는 경향이었다. 따라서 홍역이환 개에서 발생되는 수초탈락은 일차적으로 수초가 공격을 받아 파괴됨을 알 수 있었고 동시에 부위에 따라 다른 소견을 요인별로 비교하였다.