Department of Neurology, JA Toride Medical Center, Ibaraki, Japan.
J Neurol Sci. 2013 Dec 15;335(1-2):224-7. doi: 10.1016/j.jns.2013.09.005. Epub 2013 Sep 13.
Progressive supranuclear palsy-Richardson's syndrome (PSP-RS) is a neurodegenerative disease characterized by postural instability and vertical gaze palsy, but the clinical diagnosis of PSP-RS is often difficult in the early stage of the disease. A 64-year-old male experienced frequent falls, followed by dysarthria and dysphagia. Neurological examination at age 64 demonstrated vertical gaze palsy, dysarthria, dysphagia, and retropulsion. At that time, while brain MRI demonstrated no apparent abnormalities, SPECT showed the reduction of the cerebral blood flow in the thalamus as well as the medial frontal lobe cortices. The patient was diagnosed with probable PSP-RS, and died at age 70. On postmortem examination, there were abundant tuft-shaped astrocytes, neurofibrillary tangles, coiled bodies, and argyrophilic threads in the brain, establishing the diagnosis of PSP-RS. Our definite PSP-RS case suggests that thalamic hypoperfusion may provide helpful evidence to support a diagnosis of PSP-RS in the early stage of the disease.
进行性核上性麻痹-理查森综合征（PSP-RS）是一种以姿势不稳和垂直性眼球运动障碍为特征的神经退行性疾病，但 PSP-RS 的临床诊断在疾病早期通常较为困难。一名 64 岁男性出现频繁跌倒，随后出现构音障碍和吞咽困难。64 岁时的神经检查显示垂直性眼球运动障碍、构音障碍、吞咽困难和后退。当时，尽管脑 MRI 未见明显异常，但 SPECT 显示丘脑和内侧额叶皮质的脑血流减少。该患者被诊断为可能的 PSP-RS，并于 70 岁时死亡。尸检时，大脑中存在大量毛球状星形胶质细胞、神经纤维缠结、卷曲体和嗜银纤维，确立了 PSP-RS 的诊断。我们的明确 PSP-RS 病例提示，丘脑低灌注可能为支持疾病早期 PSP-RS 的诊断提供有价值的证据。
