Harris G J, Aylward E H, Peyser C E, Pearlson G D, Brandt J, Roberts-Twillie J V, Barta P E, Folstein S E
Department of Psychiatry, Tufts University, New England Medical Center, Boston, Massachusetts, USA.
Arch Neurol. 1996 Apr;53(4):316-24. doi: 10.1001/archneur.1996.00550040044013.
To examine basal ganglia dysfunction and atrophy in patients with mild to moderate Huntington's disease, with correlation of imaging measures with clinical and neuropsychological measures.
Survey study in patients with Huntington's disease and matched controls, with imaging measures being evaluated by investigators unaware of the diagnosis.
Baltimore Huntington's Disease Project, The Johns Hopkins Hospital, Baltimore, Md.
Subjects included 10 patients with mild to moderate Huntington's disease and nine healthy age-matched control subjects.
Imaging measures included single photon emission computed tomographic regional cerebral blood flow in caudate, putamen, and thalamus, and magnetic resonance imaging measures of caudate and putamen volumes and bicaudate ratios. Patients underwent neurologic and mental status examinations and neuropsychological tests.
The measure with the greatest difference between patients and control subjects was mean putamen volume, reduced 54.3% in patients, with no overlap between groups (P<.001). Of the cerebral blood flow measures, caudate showed the greatest difference (21.5% decrease; P<.001). Quantitative neurologic indexes of disease severity correlated with both putamen measures (P<.03), while Mini-Mental State Examination scores correlated with caudate volume (P<.02). Bicaudate ratio correlated with both clinical measures and was the best index of neurologic deterioration (r=.95; P<.001), while global atrophy (measured by cerebrospinal fluid percentage) was the best correlate of several neuropsychological tests, such as the Trail Making Test (r=93; P<.001).
Volumetric measurement of putamen best discriminated patients with Huntington's disease from healthy subjects. Measures of caudate atrophy or single photon emission computed tomographic measures performed less well. Neurologic decline correlated best with subcortical atrophy measured by the bicaudate ratio, but neuropsychological performance best corresponded to cerebrospinal fluid percentage, a measure of global atrophy.
研究轻至中度亨廷顿病患者的基底神经节功能障碍和萎缩情况，并将影像学测量结果与临床及神经心理学测量结果进行相关性分析。
对亨廷顿病患者及匹配的对照组进行调查研究，由不知晓诊断结果的研究人员对影像学测量结果进行评估。
马里兰州巴尔的摩市约翰霍普金斯医院巴尔的摩亨廷顿病项目组。
研究对象包括10例轻至中度亨廷顿病患者和9名年龄匹配的健康对照者。
影像学测量指标包括尾状核、壳核和丘脑的单光子发射计算机断层扫描区域脑血流量，以及尾状核和壳核体积及双侧尾状核比率的磁共振成像测量结果。患者接受了神经系统和精神状态检查以及神经心理学测试。
患者与对照组之间差异最大的测量指标是壳核平均体积，患者组壳核平均体积减少了54.3%，两组之间无重叠（P<0.001）。在脑血流量测量指标中，尾状核的差异最大（减少21.5%；P<0.001）。疾病严重程度的定量神经学指标与壳核的两项测量指标均相关（P<0.03），而简易精神状态检查得分与尾状核体积相关（P<0.02）。双侧尾状核比率与临床测量指标均相关，是神经功能恶化的最佳指标（r=0.95；P<0.001），而整体萎缩（通过脑脊液百分比测量）是多项神经心理学测试的最佳相关指标，如连线测验（r=0.93；P<0.001）。
壳核体积测量能最好地区分亨廷顿病患者与健康受试者。尾状核萎缩测量指标或单光子发射计算机断层扫描测量指标的效果较差。神经功能衰退与通过双侧尾状核比率测量的皮质下萎缩相关性最好，但神经心理学表现与作为整体萎缩指标的脑脊液百分比最为对应。
