Calderón-González P L, Parra-Rodríguez M A, Rodríguez-Martínez W, Lestayo-O'Farrill Z, Gutiérrez-Gil J, Montejo-Pujadas Y
Departamento de Neurofisiología Clínica, Instituto Nacional de Neurología y Neurocirugía, Ciudad de la Habana, Cuba.
Rev Neurol. 2004;39(1):12-7.
Steinert's myotonic dystrophy (SMD) is a systemic-type dominant autosomal disease, with variable clinical expression. Recent magnetic resonance studies conducted in patients with this disease have described the presence of lesions in the white matter of the brain and there have also been reports of a correlation between these and the presence of cognitive disorders. Nevertheless, very little work has been published about the electroencephalographic (EEG) findings in this disease.
In this study both conventional and quantitative EEG were performed on 10 patients with SMD aged between 17 and 50 years.
90% of the patients showed a posterior alpha rhythm that was disorganised but which reacted on opening and closing the eyes, as well as the presence of continuous theta activity over the base activity that was bilaterally more pronounced towards the frontal-central regions. In the quantitative analysis we observed an increase in the absolute energies for the slow bands and a decrease for the fast bands on the frequency spectrum. In most patients (80%) spectral peaks were found within the theta range of frequencies as a correlate of the slow activity observed in the conventional analysis.
It can be concluded that a quantitative EEG could be useful in the study of what, for many years, has been considered to be a 'neuromuscular' disease and that the use of other more precise methods, such as cerebral coherence and brain electrical tomography, could shed new light on the functional management of these patients.
斯坦纳特肌强直性营养不良（SMD）是一种全身性显性常染色体疾病，临床表现多样。最近对该病患者进行的磁共振研究描述了大脑白质中存在病变，也有报道称这些病变与认知障碍的存在之间存在关联。然而，关于这种疾病的脑电图（EEG）研究结果的报道却很少。
在本研究中，对10名年龄在17至50岁之间的SMD患者进行了常规脑电图和定量脑电图检查。
90%的患者显示后头部阿尔法节律紊乱，但对睁眼和闭眼有反应，并且在基础活动上存在持续的θ活动，双侧额叶中央区域更为明显。在定量分析中，我们观察到频谱上慢波带的绝对能量增加，快波带的绝对能量减少。在大多数患者（80%）中，频谱峰值出现在θ频率范围内，这与常规分析中观察到的慢活动相关。
可以得出结论，定量脑电图在研究这种多年来被认为是“神经肌肉”疾病方面可能有用，并且使用其他更精确的方法，如脑相干分析和脑电断层扫描，可能会为这些患者的功能管理提供新的线索。
