Appenzeller Simone, Chaloult Erik, Velho Paulo, de Souza Elemir Macedo, Araújo Verônica Zanardi, Cendes Fernando, Li Li Min
Neuroimaging Lab, Cidade Universitaria Zeferino Vaz CEP, 13083970 Campinas-SP, Brazil.
J Neuroimaging. 2006 Apr;16(2):154-6. doi: 10.1111/j.1552-6569.2006.00018.x.
To describe imaging findings of central nervous system involvement in patients with lipoid proteinosis.
Patients with lipoid proteinosis followed in the dermatology and neurology departments of the State University of Campinas between 2002 and 2004 were evaluated using high-resolution MRI and computerized tomography (CT).
We reviewed the charts of three patients (two women) with lipoid proteinosis with, ages ranging from 5 to 44 years. Symptomatic disease duration ranged from 2 to 39 years. MRI scans showed hypointense signal in FLAIR and T2 images in the amygdalae in two of three patients. Brain CT scans from two patients showed that MRI findings were calcifications. The calcification was more evident in patients with longer disease duration. Epilepsy was identified in one patient with longer disease duration. No other epileptogenic lesion was identified on MRI in this patient.
Lipoid proteinosis is associated with bilateral amygdalae calcification. These findings were more evident in patients with longer disease duration. Epilepsy, when present, may be related to these calcifications. Patients with lipoid proteinosis should be followed with MRI/CT in order to identify these abnormalities.
描述类脂蛋白沉积症患者中枢神经系统受累的影像学表现。
对2002年至2004年在坎皮纳斯州立大学皮肤科和神经科就诊的类脂蛋白沉积症患者进行高分辨率磁共振成像（MRI）和计算机断层扫描（CT）评估。
我们回顾了3例（2例女性）类脂蛋白沉积症患者的病历，年龄在5至44岁之间。有症状的疾病持续时间为2至39年。3例患者中有2例在MRI的液体衰减反转恢复序列（FLAIR）和T2图像上显示杏仁核低信号。2例患者的脑部CT扫描显示MRI表现为钙化。病程较长的患者钙化更明显。1例病程较长的患者被诊断为癫痫。该患者MRI未发现其他致痫性病变。
类脂蛋白沉积症与双侧杏仁核钙化有关。这些表现在病程较长的患者中更明显。癫痫（若存在）可能与这些钙化有关。类脂蛋白沉积症患者应进行MRI/CT检查以发现这些异常。
