Bjerkås I
Acta Neuropathol. 1977 Oct 10;40(2):163-9. doi: 10.1007/BF00688706.
A recently recognised progressive disorder of central myelin in Dalmatian dogs is described. The disease is probably transmitted by autosomal recessive inheritance. The symptoms started at the age of 3-6 months and were dominated by visual deficiency and/or progressive locomotive abnormalities. Pathologically, the most consistent lesions were found bilaterally in the region of the centrum semiovale. The optic nerves were also frequently affected, the basal ganglia and spinal cord were affected infrequently. The main lesions were confined to myelin, while axons and nerve cells were spared, particularly in the early stages of the lesions. Numerous phagocytes were observed which contained engulfed myelin and other lipid breakdown products. Ultrastructurally and histochemically the disease differs from previously described types of leucodystrophy.
本文描述了一种最近在大麦町犬中发现的中枢性髓鞘进行性疾病。该疾病可能通过常染色体隐性遗传传递。症状始于3至6个月龄，主要表现为视力缺陷和/或进行性运动异常。病理上，最一致的病变双侧出现在半卵圆中心区域。视神经也经常受到影响，基底神经节和脊髓很少受到影响。主要病变局限于髓鞘，而轴突和神经细胞未受影响，尤其是在病变早期。观察到大量吞噬细胞，其中含有吞噬的髓鞘和其他脂质分解产物。在超微结构和组织化学上，该疾病与先前描述的白质营养不良类型不同。
