Attig E, Jacquy J, Uytdenhoef P, Roland H
Department of Neurology, Hôpital Civil de Charleroi, Belgium.
Can J Neurol Sci. 1993 May;20(2):154-7. doi: 10.1017/s0317167100047740.
A 72-year-old man developed a very progressive neuropsychologic deficit 6 years ago, beginning with isolated visual topographic memory disturbances and visuo-constructive apraxia without additional manifestations of dementia. The syndrome worsened thereafter with the emergence of visual agnosia, simultagnosia, psychic paralysis of gaze, auditivo-verbal agnosia, and recently an amnestic syndrome with confabulation and confusion (at the end of 1989). CT scans, which remained unchanged over the years, showed mild, focal atrophic changes revealed by dilatation of the right occipital horn. His angiograms were normal. Two SPECT studies (with HMPAO measurements), performed 6 years from the onset, detected marked hypoperfusion in both parieto-occipital regions, mainly on the right side. Progressive focal degenerative disease without dementia is a relatively new syndrome, especially in cases with progressive aphasia. As noted in our patient, progressive disturbances initially localized in the right parieto-occipital region followed by posterior bilobar degeneration (pronounced on the right side) without dementia until late in the course may represent another exceptionally reported characteristic of this new syndrome. It is suggested that this variant of the Mesulam syndrome is more likely explained by progressive atrophy of the Alzheimer type.
一名72岁男性于6年前出现进行性神经心理缺陷，起初表现为孤立的视觉地形记忆障碍和视觉构建失用症，无痴呆的其他表现。此后，该综合征逐渐加重，出现视觉失认、同时失认、凝视性精神麻痹、听觉言语失认，最近还出现了伴有虚构和意识模糊的遗忘综合征（1989年底）。多年来CT扫描结果未变，显示右侧枕角扩张提示轻度局灶性萎缩改变。其血管造影正常。发病6年后进行的两项单光子发射计算机断层扫描研究（采用六甲基丙烯胺肟测量）检测到双侧顶枕叶区域明显灌注不足，主要在右侧。无痴呆的进行性局灶性退行性疾病是一种相对较新的综合征，尤其是在伴有进行性失语的病例中。正如我们的患者所示，进行性障碍最初定位于右侧顶枕叶区域，随后出现双侧后叶变性（右侧更明显），在病程晚期之前无痴呆，这可能是这种新综合征另一个罕见报道的特征。有人认为，梅苏拉姆综合征的这种变异型更可能由阿尔茨海默型进行性萎缩来解释。
