Martin J J, Ceuterick C
University of Antwerp.
Acta Neurol Belg. 1997 Jun;97(2):85-92.
A workshop on the diagnostic criteria of adult neuronal ceroid-lipofuscinosis (ANCL) or Kufs' disease represented an unique opportunity to make a critical review of the ANCL material from the files of the Born-Bunge Foundation. Our review included the clinical data when available, the light microscopic sections and the electron micrographs. When the postmortem material had not been immediately fixed in glutaraldehyde, we sampled formalin-fixed material, post-fixed it and used classical electron microscopic techniques to better define the ultrastructure of the stored products. Only one family fulfilled the criteria for ANCL Neurons and vascular smooth muscle cells contained membrane-bound inclusions with curvilinear, rectilinear and fingerprint profiles.
关于成人神经元蜡样脂褐质沉积症（ANCL）或库夫斯病诊断标准的研讨会，为批判性回顾博恩 - 邦格基金会档案中的ANCL材料提供了一个独特的机会。我们的回顾包括可获取的临床数据、光镜切片和电子显微照片。当尸检材料未立即用戊二醛固定时，我们采集福尔马林固定的材料，进行后固定，并使用经典电子显微镜技术来更好地界定储存产物的超微结构。只有一个家族符合ANCL的标准。神经元和血管平滑肌细胞含有具有曲线、直线和指纹状轮廓的膜结合内含物。
