Syka Michael, Keller Jiří, Klempíř Jiří, Rulseh Aaron M, Roth Jan, Jech Robert, Vorisek Ivan, Vymazal Josef
Department of Radiology, Na Homolce Hospital, Prague, Czech Republic; International Clinical Research Center, St. Anne´s University Hospital, Brno, Czech Republic; Institute of Experimental Medicine, Academy of Sciences of the Czech Republic, v.v.i., Prague, Czech Republic.
Department of Radiology, Na Homolce Hospital, Prague, Czech Republic; Department of Neurology, 3rd Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
PLoS One. 2015 Mar 17;10(3):e0118907. doi: 10.1371/journal.pone.0118907. eCollection 2015.
Huntington's disease (HD) is an inherited neurodegenerative disorder with progressive impairment of motor, behavioral and cognitive functions. The clinical features of HD are closely related to the degeneration of the basal ganglia, predominantly the striatum. The main striatal output structure, the globus pallidus, strongly accumulates metalloprotein-bound iron, which was recently shown to influence the diffusion tensor scalar values. To test the hypothesis that this effect dominates in the iron-rich basal ganglia of HD patients, we examined the globus pallidus using DTI and T2 relaxometry sequences. Quantitative magnetic resonance (MR), clinical and genetic data (number of CAG repeats) were obtained from 14 HD patients. MR parameters such as the T2 relaxation rate (RR), fractional anisotropy (FA) and mean diffusivity (MD) were analysed. A positive correlation was found between RR and FA (R2=0.84), between CAG and RR (R2=0.59) and between CAG and FA (R2=0.44). A negative correlation was observed between RR and MD (R2=0.66). A trend towards correlation between CAG and MD was noted. No correlation between MR and clinical parameters was found. Our results indicate that especially magnetic resonance FA measurements in the globus pallidus of HD patients may be strongly affected by metalloprotein-bound iron accumulation.
亨廷顿舞蹈症(HD)是一种遗传性神经退行性疾病,会导致运动、行为和认知功能逐渐受损。HD的临床特征与基底神经节(主要是纹状体)的退化密切相关。主要的纹状体输出结构苍白球,会大量积累与金属蛋白结合的铁,最近的研究表明这种铁会影响扩散张量标量值。为了验证这一效应在HD患者富含铁的基底神经节中占主导地位的假设,我们使用扩散张量成像(DTI)和T2弛豫测量序列对苍白球进行了检查。我们从14名HD患者那里获取了定量磁共振(MR)、临床和基因数据(CAG重复序列数量)。分析了诸如T2弛豫率(RR)、分数各向异性(FA)和平均扩散率(MD)等MR参数。发现RR与FA之间存在正相关(R2 = 0.84),CAG与RR之间存在正相关(R2 = 0.59),CAG与FA之间存在正相关(R2 = 0.44)。观察到RR与MD之间存在负相关(R2 = 0.66)。注意到CAG与MD之间存在相关趋势。未发现MR与临床参数之间存在相关性。我们的结果表明,尤其是HD患者苍白球的磁共振FA测量可能会受到与金属蛋白结合的铁积累的强烈影响。