Robain O, Ponsot G, Hulin R, Arthuis M
Arch Fr Pediatr. 1981 Jan;38(1):19-24.
The authors report the case of a 16 year-old girl with the following features: clinically, a progressive bulbar paralysis, a weakness and wasting of muscles predominantly in the upper limbs; pathologically, a severe neuronal loss in the motor nuclei of the VIIth, IXth, XIIth cranial nerves and in the anterior horns of the cervical and thoracic spinal cord, a demyelinisation of the corticospinal tracts. A classification of progressive bulbar paralysies in infants and children is proposed. In the first group, the peripheral motoneuron is the only involved. Such cases are often called Fazio-Londe disease and can be related to those cases of infantile spinal amyotrophy either of the Werdnig-Hoffmann type or, most often, of the Wolfhardt-Kugelberg type. In the second group, the corticospinal tract is also involved. Some of these cases can be included in the spinocerebellar degenerations but others, such as the case reported here, are strongly reminiscent of the adult amyotrophic lateral sclerosis.
作者报告了一名16岁女孩的病例，其具有以下特征：临床上，进行性延髓麻痹，肌肉无力及萎缩主要在上肢；病理上，第VII、IX、XII对脑神经运动核以及颈髓和胸髓前角有严重神经元丧失，皮质脊髓束脱髓鞘。本文提出了婴幼儿进行性延髓麻痹的一种分类。第一组中，仅外周运动神经元受累。此类病例常被称为法齐奥-隆德病，可能与婴儿脊髓性肌萎缩的病例相关，后者要么是韦尔尼克-霍夫曼型，要么（最常见的）是沃尔夫哈特-库格尔贝格型。第二组中，皮质脊髓束也受累。其中一些病例可归入脊髓小脑变性，但其他病例，如此处报告的病例，很容易让人联想到成人肌萎缩侧索硬化。
