Kawazawa S, Kusunose Y, Hara T, Kodama K, Nogaki H, Yamada M
No To Shinkei. 1984 Aug;36(8):737-41.
Mental symptom is one of the characteristic features in myotonic dystrophy, but documentation of mental signs including pathological changes of the brain has been imprecise. An attempt to show the involvement of the central nervous system by CT scan and EEG has been done in our department. The material consists of 14 patients 10 males and 4 females, from 15 to 50 years of age. CT scan of the 14 patients showed diffuse enlargement of lateral and third ventricles without definite atrophy of cerebral cortex and brainstem. Measurements of various parts of the ventricles showed a difference and an increase with age of values compared with that obtained from 49 neurologically normal persons (P less than 0.01). Main findings of EEG taken from 7 patients were slowing and low voltage of background activity, and 4 patients showed monotonous alpha waves. Both asymmetry and paroxysmal waves were not found. There was no significant correlation between the degree of ventricular enlargement and the frequency of the alpha activity (P greater than 0.05). Our study reveals that the ventricular enlargement is more correlated with aging than the severity of muscle involvement or the degree of mental deterioration. This fact possibly reflects a progressive nature of the pathology of the brain, especially the white matter.
精神症状是强直性肌营养不良的特征性表现之一，但包括脑部病理改变在内的精神体征记录并不准确。我们科室曾尝试通过CT扫描和脑电图来显示中枢神经系统是否受累。研究材料包括14例患者，其中男性10例，女性4例，年龄在15岁至50岁之间。14例患者的CT扫描显示侧脑室和第三脑室弥漫性扩大，大脑皮质和脑干无明确萎缩。脑室各部位的测量结果显示存在差异，与49名神经功能正常者相比，这些值随年龄增加而升高（P小于0.01）。7例患者脑电图的主要表现为背景活动减慢和低电压，4例患者出现单调的α波。未发现不对称性和阵发性波。脑室扩大程度与α活动频率之间无显著相关性（P大于0.05）。我们的研究表明，脑室扩大与衰老的相关性比与肌肉受累严重程度或精神衰退程度的相关性更大。这一事实可能反映了脑部病理学，尤其是白质病理学的渐进性本质。
