Benson D F, Davis R J, Snyder B D
Department of Neurology, UCLA School of Medicine.
Arch Neurol. 1988 Jul;45(7):789-93. doi: 10.1001/archneur.1988.00520310107024.
Five patients had progressive dementia heralded by disorders of higher visual function. All eventually developed alexia, agraphia, visual agnosia, and components of Balint's, Gerstmann's, and transcortical sensory aphasia syndromes. Memory, insight, and judgment were relatively preserved until late in the course. Predominant parieto-occipital atrophy was demonstrated on both computed tomography and magnetic resonance imaging in two of the patients; posterior circulation was normal by angiography in the three studied. To date, no pathologic specimen is available for study; speculations on the underlying pathologic condition include an atypical clinical variant of Alzheimer's disease, a lobar atrophy analogous to Pick's disease, or some previously unrecognized entity.
5例患者以高级视觉功能障碍为先兆出现进行性痴呆。最终均出现失读症、失写症、视觉失认症以及巴林特综合征、格斯特曼综合征和经皮质感觉性失语综合征的部分症状。在病程晚期之前，记忆、洞察力和判断力相对保留。2例患者的计算机断层扫描和磁共振成像均显示顶枕叶为主的萎缩；3例接受血管造影检查的患者后循环正常。迄今为止，尚无病理标本可供研究；对潜在病理状况的推测包括阿尔茨海默病的非典型临床变异型、类似于皮克病的脑叶萎缩，或某些先前未被认识的疾病实体。
