Kondo A, Suzuki K
Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill.
Clin Neuropathol. 1993 May-Jun;12(3):169-74.
Capillaries in brain biopsies obtained from patients with globoid cell leukodystrophy (GLD), adreno-leukodystrophy (ALD), Canavan's disease (spongy degeneration) and Alexander's disease were examined ultrastructurally and morphometrically. The numbers of pinocytotic vesicles were significantly increased in capillaries in the white matter of all these patients except for a patient with GLD, in comparison with those in the gray matter of each patient. Enlargement of the perivascular space (PVS) was also observed in the white matter more frequently, together with collagen fibers and cytoplasm of infiltrating cells in the patients with ALD, Canavan's disease and Alexander's disease. In a case of GLD, the enlargement of PVS was less although demyelination was as advanced as in a case of ALD. These pathological findings were correlated with the positive contrast enhancement of computerized tomography (CT) images which have been reported in these white matter disorders. It is postulated that the increase of pinocytotic vesicles and the enlargement of PVS are closely related with the disruption of blood-brain barrier BBB) in these conditions.
对从患有球状细胞脑白质营养不良（GLD）、肾上腺脑白质营养不良（ALD）、卡纳万病（海绵状变性）和亚历山大病的患者身上获取的脑活检组织中的毛细血管进行了超微结构和形态计量学检查。与每位患者灰质中的毛细血管相比，除一名GLD患者外，所有这些患者白质中的毛细血管内吞小泡数量均显著增加。在ALD、卡纳万病和亚历山大病患者的白质中，还更频繁地观察到血管周围间隙（PVS）扩大，同时伴有胶原纤维和浸润细胞的细胞质。在一例GLD患者中，尽管脱髓鞘程度与一例ALD患者相同，但PVS扩大程度较小。这些病理发现与这些白质疾病中计算机断层扫描（CT）图像上报道的阳性对比增强相关。据推测，在这些情况下，内吞小泡的增加和PVS的扩大与血脑屏障（BBB）的破坏密切相关。
