Friedman D I, Jankovic J, Rolak L A
Neurology. 1986 Dec;36(12):1590-3. doi: 10.1212/wnl.36.12.1590.
Two men, aged 25 and 33 years, had progressive hemidystonia and an arteriovenous malformation (AVM) in the contralateral cerebral hemisphere. One patient with an AVM in the posterior basal ganglia of the right hemisphere had an older brother with severe generalized dystonia. The second patient had an AVM in the left cortical and subcortical parietal area with no obvious lesion in the basal ganglia. Unlike generalized dystonia, a focal lesion is commonly found in patients with unilateral dystonia. The association of AVM-induced hemidystonia and family history of dystonia suggests that genetic predisposition may be important in some patients with hemidystonia.
两名男性,年龄分别为25岁和33岁,患有进行性偏侧肌张力障碍,且在对侧脑半球存在动静脉畸形(AVM)。一名右侧半球基底节后部患有AVM的患者有一个患有严重全身性肌张力障碍的哥哥。第二名患者左侧皮质和皮质下顶叶区域存在AVM,基底节无明显病变。与全身性肌张力障碍不同,单侧肌张力障碍患者通常可发现局灶性病变。AVM诱发的偏侧肌张力障碍与肌张力障碍家族史之间的关联表明,遗传易感性在一些偏侧肌张力障碍患者中可能很重要。