Duffy P, Mayeux R, Kupsky W
Department of Neurology, Columbia University, College of Physicians and Surgeons, New York.
Arch Neurol. 1988 Oct;45(10):1097-100. doi: 10.1001/archneur.1988.00520340051011.
"Spongy change," or vacuolar change, was observed in the neocortex of temporal and frontal lobes in four of 27 patients with clinical and pathologic characteristics of Alzheimer's disease. All four cases had the autosomal dominant form of the disease, and aside from severe dementia, all developed myoclonus and became mute before death. The degeneration observed was unlike that seen in Creutzfeldt-Jakob disease because it lacked astrocytosis and had a different distribution of lesions. Moreover, the abundance of neurofibrillary changes and senile plaques was consistent with Alzheimer's disease. None of the other 23 cases were familial, and none developed myoclonus or mutism; three of them had minor vacuolation limited to the temporal lobe. The four cases with severe spongy change described are the first, to our knowledge, in which vacuolar or spongy change has been associated with the familial form of Alzheimer's disease.
在27例具有阿尔茨海默病临床和病理特征的患者中，有4例在颞叶和额叶新皮质观察到“海绵状改变”或空泡样改变。所有4例均为常染色体显性形式的该病，除严重痴呆外，均出现肌阵挛，并在死亡前变得缄默。观察到的变性与克雅氏病不同，因为它缺乏星形细胞增生且病变分布不同。此外，神经原纤维变化和老年斑的数量与阿尔茨海默病一致。其他23例均非家族性，均未出现肌阵挛或缄默；其中3例有局限于颞叶的轻微空泡形成。据我们所知，所描述的4例严重海绵状改变是首次将空泡样或海绵状改变与家族性阿尔茨海默病相关联的病例。
