Zweig R M, Hedreen J C, Jankel W R, Casanova M F, Whitehouse P J, Price D L
Neuropathology Laboratory, Johns Hopkins University School of Medicine, Baltimore, MD 21205-2182.
Neurology. 1988 May;38(5):702-6. doi: 10.1212/wnl.38.5.702.
Examination of brains from four individuals with the clinical diagnosis of primary dystonia revealed histopathologic abnormalities in two cases. A 29-year-old man with a 15-year history of dystonia musculorum deformans (DMD) had numerous neurofibrillary tangles (NFT) and mild neuronal loss within the locus ceruleus; occasional NFT were also recognized in the substantia nigra pars compacta, pedunculopontine nucleus, and dorsal raphe nucleus. A 68-year-old man with a 35-year history of Meige syndrome had moderate-to-severe neuronal loss in several brainstem nuclei, including the substantia nigra pars compacta, locus ceruleus, raphe nuclei, and pedunculopontine nucleus. Infrequent NFT were also noted in substantia nigra. An examination of these and other brain regions in a 10-year-old boy with a 6-year history of DMD and a 50-year-old woman with a 3-year history of spasmodic torticollis did not disclose similar abnormalities.
对四名临床诊断为原发性肌张力障碍患者的大脑进行检查，结果显示其中两例存在组织病理学异常。一名患有变形性肌张力障碍（DMD）15年的29岁男性，在蓝斑内有大量神经原纤维缠结（NFT）和轻度神经元丢失；在黑质致密部、脚桥核和中缝背核中也偶尔发现NFT。一名患有Meige综合征35年的68岁男性，在几个脑干核团中存在中度至重度神经元丢失，包括黑质致密部、蓝斑、中缝核和脚桥核。在黑质中也偶尔发现NFT。对一名患有DMD 6年的10岁男孩和一名患有痉挛性斜颈3年的50岁女性的这些及其他脑区进行检查，未发现类似异常。
