摘要
目的:探讨苍白球、黑质、红核色素变性的临床和MRI特点。材料和方法:回顾性分析2例苍白球、黑质、红核色素变性的临床和MRI表现。结果:主要临床特点:少年期起病,病情缓慢进展,主要表现为锥体外系症状,均有言语障碍,其中1例有精神障碍、视神经萎缩、步态姿势异常和双侧巴宾斯基征( + ) ;T2WI均表现为苍白球、黑质等部位对称性低信号,其中1例为“虎眼征”。结论:根据临床和MRI特点可以准确诊断HSD。
Purpose: To explore the clinical and MRI characteristics of Hallervorden-Spatz disease (HSD). Materials and Methods: The clinical and MRI features of 2 patients with HSD were analysed retrospectively. Results: The major clinical features including juvenile onset with slow progression, extrapyramidal syndromes and dysarthria. One case had Gait or postural difficulties, pathologic reflex, optic atrophy and psychiatric symptoms. Significant low signal intensity on T2 WI localized in bilateral globus pallidus and pars compacta of the substantia nigra in two patients, and the 'eye of the tiger' sign in one patient. Conclusion: HSD can be diagnosed on the basis of clinical and MRI features.
出处
《中国医学影像学杂志》
CSCD
2005年第3期167-169,共3页
Chinese Journal of Medical Imaging
基金
国家 8 63高技术研究发展计划资助项目 ( 2 0 0 2BA71 1A0 7
2 0 0 4AA2 2 70 4 0 )
国家自然科学基金 ( 30 370 5 1 5 )
高等学校博士学科点专项科研基金 ( 2 0 0 2 0 5 330 2 4 )
