Humphreys P, Kaufmann W E, Galaburda A M
Dyslexia Research Laboratory, Charles A. Dana Research Institute, Boston, MA.
Ann Neurol. 1990 Dec;28(6):727-38. doi: 10.1002/ana.410280602.
Brains from male cases with dyslexia show symmetry of the planum temporale and predominantly left-sided cerebrocortical microdysgenesis. We now report on three women with dyslexia. In all brains, the planum temporale was again symmetrical. Also, in two of the brains, multiple foci of cerebrocortical glial scarring were present. In both women, many of the scars were myelinated, suggesting origination during late intrauterine or early postnatal life. In one, scars were mainly left perisylvian and involved portions of the vascular border zone of the temporal cortex. In the other, scars were more numerous and occurred in the border zone of the anterior, middle, and posterior cerebral arteries symmetrically. All three cases showed to a variable extent brain warts, molecular layer ectopias, and focal architectonic dysplasia identical to those seen in the male cases. Two women had primary brain neoplasms, an oligodendroglioma and a low-grade astrocytoma, respectively, and two women showed small angiomas. Reexamination of previously reported male cases disclosed one with myelinated glial scars. Two control brains with asymmetrical plana temporale showed myelinated glial scars as well. The significance of the anatomical findings is discussed, and possible etiological factors are considered with known effects of autoimmune diseases on the nervous system.
患有诵读困难症的男性病例的大脑显示颞平面对称,且主要为左侧大脑皮质微发育异常。我们现在报告三例患有诵读困难症的女性。在所有大脑中,颞平面再次呈现对称。此外,在其中两个大脑中,存在多个大脑皮质胶质瘢痕灶。在这两名女性中,许多瘢痕都有髓鞘形成,提示其起源于子宫内晚期或出生后早期。其中一名女性,瘢痕主要位于左侧外侧裂周围,累及颞叶皮质血管边缘带的部分区域。另一名女性的瘢痕更多,对称地出现在大脑前、中、后动脉的边缘带。所有三例在不同程度上均表现出脑疣、分子层异位和局灶性结构发育异常,与男性病例所见相同。两名女性分别患有原发性脑肿瘤,一个是少突胶质细胞瘤,另一个是低级别星形细胞瘤,还有两名女性有小血管瘤。对先前报告的男性病例进行重新检查发现一例有髓鞘形成的胶质瘢痕。两个颞平面不对称的对照大脑也有髓鞘形成的胶质瘢痕。本文讨论了解剖学发现的意义,并结合自身免疫性疾病对神经系统的已知影响考虑了可能病因。