Censori B, Provinciali L, Danni M, Chiaramoni L, Maricotti M, Foschi N, Del Pesce M, Salvolini U
Institute of Neurological Diseases, University of Ancona, Italy.
Acta Neurol Scand. 1994 Sep;90(3):211-7. doi: 10.1111/j.1600-0404.1994.tb02708.x.
A prospective, case-control study was carried out on 25 patients with myotonic dystrophy (MyD) and 25 healthy subjects using brain magnetic resonance imaging (MRI). The frequency and severity of white matter hyperintense lesions (WMHL) and brain atrophy in MyD patients were compared with their clinical features and cognitive impairment using an extensive neuropsychological battery. Eighty-four per cent of MyD patients showed WMHL, compared with 16% of controls (p < 0.0001). These lesions involved all cerebral lobes, without hemispheric prevalence. Twenty-eight per cent of MyD patients also showed particular WMHL at their temporal poles. Myotonic patients had significantly more cortical atrophy than controls. No relationship between atrophy and WMHL was found on the MRI scans. The extent of brain abnormalities (WMHL or atrophy) was not correlated to age, disease duration, physical disability or severity of neuropsychological impairment. Central nervous system abnormalities revealed by MRI appear to be an almost constant feature of MyD, but they are not found to be related to clinical or cognitive parameters. Their nature is still unclear: some of them, located at the temporal poles, seem to be characteristic of the disease, while others small, diffuse WMHLs, similar to the age related alterations revealed by MRI occurring during young and adult age in MyD patients.
一项前瞻性病例对照研究对25例强直性肌营养不良（MyD）患者和25名健康受试者进行了脑磁共振成像（MRI）检查。使用一套广泛的神经心理学测试组，将MyD患者白质高信号病变（WMHL）和脑萎缩的频率及严重程度与其临床特征和认知障碍进行了比较。84%的MyD患者出现WMHL，而对照组为16%（p<0.0001）。这些病变累及所有脑叶，无半球优势。28%的MyD患者在颞极也出现了特定的WMHL。强直性肌营养不良患者的皮质萎缩明显多于对照组。在MRI扫描中未发现萎缩与WMHL之间存在关联。脑异常（WMHL或萎缩）的程度与年龄、病程、身体残疾或神经心理学损害的严重程度无关。MRI显示的中枢神经系统异常似乎是MyD几乎恒定的特征，但未发现它们与临床或认知参数有关。其性质仍不清楚：其中一些位于颞极的病变似乎是该疾病的特征，而其他一些小的、弥漫性的WMHL，类似于MyD患者在青年和成年期MRI显示的与年龄相关的改变。
