Kurata Tomoko, Kametaka Satsuki, Ohta Yasuyuki, Morimoto Nobutoshi, Deguchi Shoko, Deguchi Kentaro, Ikeda Yoshio, Takao Yoshiki, Ohta Taisei, Manabe Yasuhiro, Sato Shuhei, Abe Koji
Department of Neurology, Okayama University Graduate School of Medicine and Dentistry, Japan.
Intern Med. 2011;50(22):2775-81. doi: 10.2169/internalmedicine.50.5954. Epub 2011 Nov 15.
Because it is often difficult to precisely diagnose and distinguish progressive supranuclear palsy (PSP) from corticobasal degeneration (CBD), multiple system atrophy-parkinsonism (MSA-P) and Parkinson's disease (PD) at the onset of the disease, we compared the patients and clarified the features of these diseases.
We compared 77 PSP, 26 CBD, 26 MSA-P and 166 PD patients from clinical and imaging points of view including cerebral blood flow (CBF) in the frontal eye field.
The clinical characteristics of PSP were supranuclear gaze disturbance, optokinetic nystagmus (OKN) impairment and falls at the first visit. On head MRI, midbrain tegmentum atrophy was much more frequently detected in PSP than in all of the other groups. Heart-to-mediastinum average count ratio (H/M) in iodine-123 meta-iodobenzyl guanidine ((123)I-MIBG) myocardial scintigraphy was not decreased in PSP, CBD, MSA-P and PD-Yahr 1 (-1), but patients of PD-2, 3, 4 and 5 showed a significant decrease compared with the PSP group. The CBF in the left frontal eye field of PD-3 group and that in right frontal eye field of PD-3 and PD-4 groups were lower than that of PSP group, although other groups showed a tendency without a significant decrease compared with PSP group.
PSP is distinguishable from CBD, MSA-P and PD even at the early stage with extra-ocular movement (EOM) disturbance, falls, atrophy of the midbrain tegmentum, and H/M in (123)I-MIBG myocardial scintigraphy, and the reduction of CBF in area 8 could serve as a supplemental diagnostic method for distinguishing PSP from PD-3 or PD-4.
由于在疾病发作时，通常难以准确诊断并区分进行性核上性麻痹（PSP）与皮质基底节变性（CBD）、多系统萎缩-帕金森型（MSA-P）和帕金森病（PD），我们对患者进行了比较并明确了这些疾病的特征。
我们从临床和影像学角度，包括额眼区脑血流量（CBF），对77例PSP患者、26例CBD患者、26例MSA-P患者和166例PD患者进行了比较。
PSP的临床特征为首次就诊时出现核上性凝视障碍、视动性眼球震颤（OKN）损害和跌倒。在头部MRI上，PSP中脑被盖萎缩的检出频率远高于所有其他组。PSP、CBD、MSA-P和PD-Yahr 1期（-1）患者的碘-123间碘苄胍（（123）I-MIBG）心肌闪烁显像中心脏与纵隔平均计数比（H/M）未降低，但PD-2、3、4和5期患者与PSP组相比显著降低。PD-3组左侧额眼区的CBF以及PD-3和PD-4组右侧额眼区的CBF低于PSP组，尽管其他组与PSP组相比有降低趋势但无显著差异。
即使在早期，PSP也可通过眼外肌运动（EOM）障碍、跌倒、中脑被盖萎缩、（123）I-MIBG心肌闪烁显像中的H/M以及8区CBF降低与CBD、MSA-P和PD相区分，8区CBF降低可作为区分PSP与PD-3或PD-4的补充诊断方法。
