Sahin Neslin, Solak Aynur, Genc Berhan, Kulu Ugur
1 Department of Radiology, 2 Department of Neurology, Sifa University School of Medicine, Izmir, Turkey.
Quant Imaging Med Surg. 2015 Aug;5(4):628-32. doi: 10.3978/j.issn.2223-4292.2015.04.01.
Fahr disease (FD) is a well-defined rare neurodegenerative disease that is characterized by idiopathic bilateral symmetric extensive striopallidodentate calcifications. The patients may present with diverse manifestations, most commonly movement disorder, cognitive impairment, and ataxia. Computed tomography (CT) is considered to be critical for accurate diagnosis because it is difficult to reliably identify calcifications by routine magnetic resonance imaging (MRI). Susceptibility-weighted imaging (SWI) is a relatively new 3D gradient-echo (GE) MR sequence with special phase and magnitude processing. SWI phase images can recognize calcifications definitively with higher sensitivity compared to other MRI sequences. In this article, we present two cases of FD with different manifestations and neuroimaging in different age groups and genders, which were diagnosed by SWI and confirmed with CT, and we discuss the contribution of SWI in the diagnosis of FD. In conclusion, we suggest integrating SWI with MRI protocol to identify calcifications in suspicion of neurodegenerative disorders.
Fahr病（FD）是一种明确的罕见神经退行性疾病，其特征为特发性双侧对称性广泛的纹状体苍白球齿状核钙化。患者可能出现多种表现，最常见的是运动障碍、认知障碍和共济失调。计算机断层扫描（CT）被认为对准确诊断至关重要，因为通过常规磁共振成像（MRI）很难可靠地识别钙化。磁敏感加权成像（SWI）是一种相对较新的三维梯度回波（GE）磁共振序列，具有特殊的相位和幅度处理。与其他MRI序列相比，SWI相位图像能够以更高的灵敏度明确识别钙化。在本文中，我们介绍了两例不同年龄组和性别的具有不同表现和神经影像学特征的FD病例，这些病例通过SWI诊断并经CT证实，我们还讨论了SWI在FD诊断中的作用。总之，我们建议将SWI纳入MRI检查方案以识别疑似神经退行性疾病中的钙化。
