Department of Neurosurgery, Charité University Clinic, Berlin, Germany.
Department of Neurosurgery, Charité University Clinic, Berlin, Germany,
Stereotact Funct Neurosurg. 2021;99(4):351-362. doi: 10.1159/000512599. Epub 2021 Jan 20.
The pathophysiology of dystonia is poorly understood. As opposed to secondary forms of dystonia, primary dystonia has long been believed to lack any neuroanatomical substrate. During trajectory planning for DBS, however, conspicuous T2-hyperinstensive signal alterations (SA) were registered within the target region, even in young patients, where ischemia is rare.
Fifty MRIs of primary dystonia patients scheduled for DBS were analyzed. Total basal ganglia (BG) volumes, as well as proportionate SA volumes, were measured and compared to 50 age-matched control patients.
There was a 10-fold preponderance of percentaged SA within the globus pallidus (GP) in dystonia patients. The greatest disparity was in young patients <25 years. Also, total BG volume differences were observed with larger GP and markedly smaller putamen and caudate in the dystonia group.
BG morphology in primary dystonia differed from a control population. Volume reductions of the putamen and caudate may reflect functional degeneration, while volume increases of the GP may indicate overactivity. T2-hyperintensive SA in the GP of young primary dystonia patients, where microvascular lesions are highly unlikely, are striking. Their pathogenic role remains unclear.
肌张力障碍的病理生理学知之甚少。与继发性肌张力障碍相反，原发性肌张力障碍长期以来被认为缺乏任何神经解剖学基础。然而，在 DBS 的轨迹规划过程中，即使在年轻患者中，也会在目标区域内记录到明显的 T2 高信号改变（SA），而在年轻患者中，缺血很少见。
分析了 50 例原发性肌张力障碍患者的 MRI，这些患者计划接受 DBS 治疗。测量并比较了总基底节（BG）体积和比例 SA 体积与 50 名年龄匹配的对照患者。
肌张力障碍患者的苍白球（GP）中 SA 的百分比呈 10 倍优势。在<25 岁的年轻患者中差异最大。此外，还观察到总 BG 体积差异，肌张力障碍组的 GP 更大，而纹状体和尾状核明显更小。
原发性肌张力障碍的 BG 形态与对照组不同。壳核和尾状核的体积减少可能反映了功能退化，而 GP 的体积增加可能表明过度活跃。在年轻的原发性肌张力障碍患者中，苍白球内出现 T2 高信号 SA，这在微血管病变中极不可能出现，这令人震惊。它们的致病作用仍不清楚。
