Reusche E
Institute of Pathology, Laboratory of Neuropathology, Medical University, Lübeck, Germany.
Acta Neuropathol. 1997 Dec;94(6):612-6. doi: 10.1007/s004010050757.
A 72-year-old female demonstrated neurofibrillary changes of Alzheimer's disease (AD) as well as morphological features of dialysis-associated encephalopathy (DAE). This unique autopsy case was selected from a group of 127 patients with a history of long-term hemodialysis. Silver-stained paraffin sections revealed hall-marks of DAE with characteristic fine-granular aluminium-containing inclusions in the cytoplasm of plexus epithelia, cortical glia and neurons. Additionally, neurofibrillary tangles (NFT) and neuropil threads of the AD-type were demonstrated, consisting of paired helical filaments. No beta/A4 amyloid deposits were present. AD changes have been interpreted as age-related phenomena. Concerning the role of aluminium (Al) in AD, our morphological findings indicate that Al-induced DAE changes develop via different pathogenetic pathways compared to AD. Transferrin receptors stained positively in choroid plexus and neurons, in glial cells of the gray and white matter, in cerebellar (DAE-negative) Purkinje and granule cells as well as in AD-type NFT (and additionally in NFT of AD controls). This points to the ubiquitous distribution of transferrin receptors rather than to specific patterns in DAE and/or AD. Laser microprobe mass analysis indicated high Al-related signals at m/z 27, confirming the increase of Al in neurons with DAE. In contrast to the former clinically endemic and lethal dialysis encephalopathy, caused by massive Al-uptake by non-deionized dialysates, the term DAE comprises a morphological description in psychiatrically mostly unconspicious patients. It is characterized by the storage of argyrophilic degradation products of lysosomal-derived exclusively intracytoplasmic Al-containing inclusions, nowadays predominantly caused by long-term ingestion of Al-containing drugs in hemodialysis.
一名72岁女性表现出阿尔茨海默病（AD）的神经原纤维变化以及透析相关性脑病（DAE）的形态学特征。这个独特的尸检病例是从127例有长期血液透析史的患者中挑选出来的。银染石蜡切片显示出DAE的特征，即丛状上皮、皮质神经胶质细胞和神经元的细胞质中有特征性的细颗粒含铝包涵体。此外，还发现了AD型神经原纤维缠结（NFT）和神经毡丝，由双螺旋丝组成。未发现β/A4淀粉样蛋白沉积。AD变化被解释为与年龄相关的现象。关于铝（Al）在AD中的作用，我们的形态学发现表明，与AD相比，Al诱导的DAE变化通过不同的致病途径发展。转铁蛋白受体在脉络丛和神经元、灰质和白质的神经胶质细胞、小脑（DAE阴性）浦肯野细胞和颗粒细胞以及AD型NFT（以及AD对照的NFT中）呈阳性染色。这表明转铁蛋白受体分布广泛，而非在DAE和/或AD中有特定模式。激光微探针质量分析表明，在m/z 27处有高Al相关信号，证实了DAE神经元中Al含量增加。与以前由非去离子透析液大量摄入Al引起的临床地方性致死性透析脑病不同，术语DAE包括对精神状态大多无明显异常患者的形态学描述。其特征是溶酶体衍生的嗜银降解产物仅在胞质内含铝包涵体中蓄积，如今主要由血液透析中长期摄入含铝药物引起。
