REACTIONS OF VESSEL WALLS AND BRAIN PARENCHYMA TO THE ACCUMULATION OF GAUCHER CELLS IN THE NORRBOTTNIAN TYPE (TYPE-III) OF GAUCHER DISEASE
REACTIONS OF VESSEL WALLS AND BRAIN PARENCHYMA TO THE ACCUMULATION OF GAUCHER CELLS IN THE NORRBOTTNIAN TYPE (TYPE-III) OF GAUCHER DISEASE
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DOI：
10.1007/bf00687792
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发表时间：
1988-01-01
影响因子：
12.7
通讯作者：
SOURANDER, P
中科院分区：
文献类型：
--
作者：
CONRADI, NG;KALIMO, H;SOURANDER, P
Norrbottnian型高谢病患儿行脾切除术后，血液中葡萄糖神经酰胺水平升高，神经和精神状态受损。与未切除脾的病例相比，血液中的高水平与脑部血管周围高雪氏细胞中葡萄糖神经酰胺的积累增加有关。高雪氏细胞周围可见神经元丢失，脑实质轻度脱髓鞘。对4例Norrbottnian型高谢病患者的脑进行免疫组织化学染色，以进一步确定血管周围的高雪氏细胞的特征，并观察血管壁和脑实质对高雪氏细胞积聚的反应。血管周围储存细胞呈颗粒状，胞壁酶和α1-抗凝乳杆菌胰凝乳酶抗体染色，证实为单核-巨噬细胞系统的血源性巨噬细胞。高谢氏细胞含有血浆蛋白抗血清阳性物质，强烈提示在高谢病中大分子(包括葡萄糖神经酰胺)可从血液中逸出并被巨噬细胞摄取。储存细胞周围有网状蛋白网络，网状蛋白被III型、IV型胶原和层粘连蛋白抗血清染色。浸润物被基底膜蛋白、IV型胶原和层粘连蛋白抗血清强阳性的膜包围。高谢氏细胞袖带周围基膜的形成可能构成了一种保护大脑实质免受外来细胞侵袭的现象。神经丝抗血清可显示局灶性神经元丢失，但仅有少量轴突丢失。抗胶质纤维酸性蛋白染色显示高雪细胞周围的脑实质有明显的星形胶质细胞增生。在先前显示血液中葡萄糖神经酰胺水平较高的两个病例中，星形胶质细胞的血浆蛋白呈阳性，表明血浆蛋白进入大脑；这在未切除脾的病例中未见。Norrbottnian型Gaucher病脾切除后神经症状和智力低下的增加，可能是Gaucher细胞浸润物附近的低级别组织损伤的叠加效应。
Splenectomy in children with the Norrbottnian type of Gaucher disease is followed by increased blood levels of glucosylceramide and impaired neurological and mental status. High blood levels were associated with an increased accumulation of glucosylceramide in perivascular Gaucher cells in the brain compared to non-splenectomised cases. Surrounding the Gaucher cell infiltrates there is loss of neurons and slight demyelination in the brain parenchyma. The brains of four cases with the Norrbottnian type of Gaucher disease were examined by immunohistochemical strains in an attempt to further characterize the perivascular Gaucher cells and to examine the reactions of the vessel walls and brain parenchyma to the accumulation of Gaucher cells. The perivascular storage cells showed granular staining with antibodies to muramidase and .alpha.1-anti-chymotrypsin confirming that they are blood-derived macrophages belonging to the monocyte-macrophage system. The Gaucher cells contained material positive for antisera to plasma proteins strongly suggesting that large molecules (including glucosylceramide) can escape from the blood and be taken up by the macrophges in Gaucher disease. The storage cells were surrounded by a reticulin network stained by antisera to collagen type III, type IV and laminin. The infiltrates were bounded from the brain parenchyma by a membrane strongly positive with antiserum for the basal lamina protein collagen type IV and laminin. The formation of a basal lamina around the Gaucher cell cuffs probably constitutes a protective phenomenon governing the brain parenchyma against the foreign cells. A focal loss of neurons but only minor loss of axons could be demonstrated with the antiserum to neurofilament. The brain parenchyma surrounding the Gaucher cell infiltrates showed marked astrogliosis in the anti-glial fibrillary acidic protein stain. In the two cases previously shown to have higher blood levels of glucosylceramide there were astrocytes positive for plasma proteins indicating passage of plasma proteins into the brain; this was not seen in the non-splenectomised cases. The additive effect of low-grade tissue damage in the vicinity of the Gaucher cell infiltrates is probably enough to explain the increased neurological symptoms and mental retardation following splenectomy in the Norrbottnian type of Gaucher disease.