Ghatak N R
Acta Neuropathol. 1978 Jan 19;41(1):1-7. doi: 10.1007/BF00689549.
The spinal nerve roots were studied in two siblings with Werdnig-Hoffmann disease (WHD). An invasion by fibrous astrocytes was consistently seen along the atrophic ventral roots, more extensively in the older sibling. The parallel glial processes extended in discrete bundles and were always enclosed by a basal lamina. Loss of unmyelinated axons and probable glial extension along unmyelinated fibers were also seen. The astrocytic processes with abundant microtubules and prominent junctional devices resembled those of the subpial region, which appeared to have gained access into the ventral roots following axonal degeneration. The present observations suggest that such glial migration, although apparently unique in WHD, is a secondary phenomenon and fails to resolve the issue as to whether neuronal degeneration or an injury to the nerve roots is the primary event in this disorder.
对两名患有韦尼克-霍夫曼病（WHD）的兄弟姐妹的脊神经根进行了研究。在萎缩的腹侧神经根周围始终可见纤维性星形胶质细胞的侵入，年龄较大的兄弟姐妹中更为广泛。平行的神经胶质突起呈离散束状延伸，且总是被基膜包围。还观察到无髓轴突的丢失以及沿无髓纤维可能的神经胶质延伸。具有丰富微管和明显连接装置的星形胶质细胞突起类似于软膜下区域的突起，后者似乎在轴突退变后进入了腹侧神经根。目前的观察结果表明，这种神经胶质迁移虽然在WHD中显然是独特的，但却是一种继发性现象，无法解决神经元退变或神经根损伤是否是该疾病主要事件的问题。
