Gómez-Tortosa E, del Barrio A, Barroso T, García Ruiz P J
Department of Neurology, Fundación Jiménez Díaz, Madrid, Spain.
J Neurol. 1996 Mar;243(3):286-92. doi: 10.1007/BF00868528.
Deficits in visual processing are early cognitive abnormalities in patients with Huntington's disease (HD) and may be found in presymptomatic gene carriers. We investigated the nature and evolution of deficits in visual processing in HD, and whether subtle deficits could be recognized by formal testing in asymptomatic carriers. We studied 35 patients with HD in stages 1-3 of functional disability, and 26 symptom-free relatives at 50% risk for the disease. We administered the Mini Mental State Examination to assess overall cognitive function and tests to assess visuospatial skills such as visual attention and ocular scanning (Cancellation Task and Line Bisection Test), visuoconstructive abilities (Copy of Rey's Complex Figure), and visuoperception (Hooper Visual Organization Test). The group at risk comprised 15 asymptomatic carriers (AC) and 11 non-carriers (NC) and was assessed by investigators blinded to gene status. HD patients were impaired in most of the tasks compared with AC and NC, and the scores declined steadily from stage 1 to 3. However, the difference between patients in stage 1 of HD and AC and NC in most of the tasks was not significant. Only the Hooper Test, which requires complex visual integration, was highly discriminative of early symptomatic from asymptomatic carriers (P < 0.05). There were no significant differences between AC and NC in any of the tasks. We conclude deficits in visual processing develop with other manifestations of the disease and are not significant on formal testing at presymptomatic stages; also, early visual deficits in HD seem to be related to disorders in complex visual processing.
视觉加工缺陷是亨廷顿舞蹈症（HD）患者早期的认知异常，且在症状前基因携带者中也可能出现。我们研究了HD患者视觉加工缺陷的本质和演变，以及无症状携带者能否通过正式测试识别出细微缺陷。我们研究了35名处于功能残疾1 - 3期的HD患者，以及26名患病风险为50%的无症状亲属。我们进行了简易精神状态检查以评估整体认知功能，并进行了一些测试来评估视觉空间技能，如视觉注意力和眼部扫描（划消测验和直线二等分测验）、视觉构建能力（复制雷氏复杂图形）和视觉感知（胡珀视觉组织测验）。风险组包括15名无症状携带者（AC）和11名非携带者（NC），由对基因状态不知情的研究人员进行评估。与AC和NC相比，HD患者在大多数任务中都存在受损情况，且分数从1期到3期稳步下降。然而，HD 1期患者与AC和NC在大多数任务上的差异并不显著。只有需要复杂视觉整合的胡珀测验能够高度区分早期有症状携带者和无症状携带者（P < 0.05）。AC和NC在任何任务中均无显著差异。我们得出结论，视觉加工缺陷随着疾病的其他表现而发展，在症状前阶段的正式测试中并不显著；此外，HD早期的视觉缺陷似乎与复杂视觉加工障碍有关。
