Perticoni G F, Cantisani T A, Fisher H
Ital J Neurol Sci. 1983 Apr;4(1):107-11. doi: 10.1007/BF02043448.
The case of a progressive bulbar paresis in a nine and a half year old child is reported. The first symptoms were present at birth; however, the subsequent evolution was very low. Lesion of the motor nuclei of the V, VII, IX, XII, cranial nerves was evident on electromyographic investigation. Damage to the acoustic brain stem pathway was documented by the brain stem evoked potentials although audiometry was normal. No other neuronal systems or districts appeared to be damaged. The case suggests Fazio-Londe disease, although the involvement (albeit partial) of the auditory pathways recalls Van Laere syndrome. This supports the view that motor neuron disease in infancy is not an autonomous entity but a variant in a wide spectrum of progressive neuronal diseases.
报告了一名9岁半儿童进行性延髓麻痹的病例。最初症状在出生时就已出现；然而，随后的病情进展非常缓慢。肌电图检查显示，第V、VII、IX、XII对脑神经的运动核有病变。尽管听力测定正常，但脑干诱发电位记录了听觉脑干通路的损伤。没有其他神经元系统或区域似乎受损。该病例提示为法齐奥-隆德病，尽管听觉通路的受累（尽管是部分受累）使人联想到范拉尔综合征。这支持了这样一种观点，即婴儿期运动神经元病不是一个独立的实体，而是广泛的进行性神经元疾病谱中的一种变异。
