Engel P A, Vinters H V, Grunnet M
Department of Medicine, University of Connecticut School of Medicine, Farmington.
J Geriatr Psychiatry Neurol. 1992 Oct-Dec;5(4):200-9. doi: 10.1177/002383099200500404.
Atypical dementias confront the adequacy of current diagnostic concepts. The two patients with atypical dementia syndromes described here shared common postmortem features of numerous neocortical neuritic (senile) plaques and microvascular amyloid, sparing of hippocampus and substantia nigra, and the virtual absence of neurofibrillary tangles. Microscopically, the two differed only by the presence of a few subcortical Lewy bodies in case 1. These similar morphologic features were associated with dramatically different clinical presentations. In the first patient, visual hallucinations, Capgras' syndrome, cognitive slowing, myoclonus, parkinsonism, and primitive reflexes evolved over 3 years. Memory and language were relatively spared. In the second, dysphagia, nonfluent aphasia, hypophonia, motor perseveration, and a severe disorder of attention developed during this 18-month illness. At autopsy, an unrecognized colon malignancy was found. Despite high neuritic plaque counts in cortex, neither the clinical nor the pathologic criteria for Alzheimer's disease adequately describe either case. The cases will be examined first as clinical, then as neuropathologic, entities. From this approach, we conclude that a specific clinical dementia syndrome may be expressed by several neuropathologic "diseases" and that a variety of clinical syndromes may represent a single neuropathologic diagnosis. This strategy identifies a conceptual dichotomy between Alzheimer's syndrome and postmortem Alzheimer's disease. Meticulous clinical and neuropathologic observation is essential in advancing an understanding of the relationship between the two.
非典型性痴呆对当前的诊断概念提出了挑战。本文描述的两名患有非典型性痴呆综合征的患者，在死后具有共同特征：新皮质神经炎性（老年）斑块和微血管淀粉样蛋白较多，海马体和黑质未受影响，几乎不存在神经原纤维缠结。在显微镜下，二者的区别仅在于病例1存在一些皮质下路易小体。这些相似的形态学特征却伴有截然不同的临床表现。首例患者在3年中逐渐出现视幻觉、卡普格拉综合征、认知迟缓、肌阵挛、帕金森症和原始反射，记忆和语言功能相对保留。第二例患者在18个月的病程中出现吞咽困难、非流畅性失语、声音低微、动作持续和严重的注意力障碍，尸检时发现了未被识别的结肠恶性肿瘤。尽管皮质中的神经炎性斑块数量较多，但无论是阿尔茨海默病的临床标准还是病理标准，都无法充分描述这两个病例。这些病例将首先作为临床实体进行检查，然后作为神经病理实体进行检查。通过这种方法，我们得出结论：一种特定的临床痴呆综合征可能由几种神经病理“疾病”表现出来，而多种临床综合征可能代表单一的神经病理诊断。这一策略揭示了阿尔茨海默综合征与死后阿尔茨海默病之间的概念性二分法。细致的临床和神经病理观察对于加深对二者关系的理解至关重要。
