Peiffer J
Acta Neuropathol. 1982;56(2):87-92. doi: 10.1007/BF00690578.
A new family with the Gerstmann-Sträussler type of subacute spongiform encephalopathy is described. Atactic symptoms, dysarthrias, and personality changes characterized the clinical course. The clinical pattern of a father and his two sons was very similar. They had been in contact with sheep by occupation. A rapidly progressing demyelinating disease (transitional diffuse-multiple sclerosis) occurred in the same family, as well as numerous cases of carcinoma. Morphologically, the Sträussler type can be differentiated from other subacute spongy encephalopathies by the occurrence of Kuru-Plaques and numerous multicentric floccular plaques both in the cerebral and cerebellar cortex, basal ganglia, and white matter.
本文描述了一个患有格斯特曼-施特劳斯勒型亚急性海绵状脑病的新家族。共济失调症状、构音障碍和人格改变是其临床病程的特征。一位父亲及其两个儿子的临床症状非常相似。他们因职业原因与羊有接触。同一家庭中还出现了一种快速进展的脱髓鞘疾病（过渡性弥漫性多发性硬化症）以及众多癌症病例。在形态学上，施特劳斯勒型可通过在大脑和小脑皮质、基底神经节及白质中出现库鲁斑和众多多中心絮状斑与其他亚急性海绵状脑病相鉴别。
