Lazzarino L G, Nicolai A
Divisione Neurologica, Ospedale Civile di Gorizia.
Acta Neurol (Napoli). 1991 Feb;13(1):25-30.
The authors report the case of a 32-year-old woman who developed symptoms and signs possible manifestations of a familial motor neuron disease with left sided pyramidal signs and marked wasting and weakness in the ipsilateral upper girdle, progressively extended to the contralateral upper limb. The CT-scan showed only frontal cortical atrophy. MRI disclosed a restricted area of increased signal intensity in the centrum semiovale of the subcortical white matter of the right prefrontal cortex. This young woman did not disclose any risk factors for cerebrovascular diseases nor other disorders of the CNS; therefore the authors are of the opinion that the white matter changes observed on MRI are not occasional findings, but are related to the pathologic process occurring in consequence of her neurodegenerative disorder (possible ALS).
作者报告了一例32岁女性病例，该患者出现了可能是家族性运动神经元病的症状和体征，表现为左侧锥体束征，同侧上肢带明显萎缩和无力，并逐渐扩展至对侧上肢。CT扫描仅显示额叶皮质萎缩。MRI显示右前额叶皮质皮质下白质半卵圆中心有一个信号强度增加的受限区域。这位年轻女性未发现任何脑血管疾病或其他中枢神经系统疾病的危险因素；因此，作者认为MRI上观察到的白质变化并非偶然发现，而是与她的神经退行性疾病（可能为肌萎缩侧索硬化症）所致的病理过程有关。
