Toyoshima Y, Wakabayashi K, Nagai H, Takahashi H
Department of Pathology, Brain Research Institute, Niigata University, Japan.
No To Shinkei. 1999 Jan;51(1):59-63.
We report a 75-year-old man who presented with parkinsonism, dementia and vertical gaze palsy. Postmortem examination revealed frontoparietal atrophy and nigral depigmentation. The temporal lobe was well preserved. Histologically, the affected cerebral cortex showed vacuolation and neuronal loss in layers 2 and 3. The substantia nigra and globus pallidus were also affected. There was gliosis in the thalamic medial nuclei and midbrain tegmentum. Neither ballooned neurons nor taupositive, argyrophilic inclusions could be found. Moreover, there was no Alzheimer pathology manifested by senile plaques and neurofibrillary tangles. In fact, the clinical and pathological features of the present case were consistent with those observed previously in two other cases reported elsewhere (Kawasaki et al. Acta Neuropathol 91: 140, 1996). In these cases, corticobasal degeneration (CBD) and frontotemporal dementia (FTD) were considered to be the principal entities in the differential diagnosis. However, we believe that these three cases, including the present one, were not examples of CBD or FTD, since neither ballooned neurons nor tau abnormalities were present, and the precentral gyrus was the area most severely involved, the temporal lobes being obviously spared. Therefore we considered that they might represent a hitherto unrecognized new disease entity.
我们报告了一名75岁男性，其表现为帕金森综合征、痴呆和垂直凝视麻痹。尸检显示额顶叶萎缩和黑质色素脱失。颞叶保存完好。组织学上，受累的大脑皮质在第2层和第3层显示空泡形成和神经元丢失。黑质和苍白球也受到影响。丘脑内侧核和中脑被盖有胶质增生。未发现气球样神经元或tau阳性、嗜银性包涵体。此外，未出现由老年斑和神经原纤维缠结所表现的阿尔茨海默病病理学特征。事实上，本病例的临床和病理特征与之前在其他地方报道的另外两例病例中观察到的特征一致（Kawasaki等人，《神经病理学学报》91: 140, 1996）。在这些病例中，皮质基底节变性（CBD）和额颞叶痴呆（FTD）被认为是鉴别诊断中的主要疾病。然而，我们认为这三例病例，包括本病例，并非CBD或FTD的实例，因为既不存在气球样神经元也不存在tau异常，且中央前回是受累最严重的区域，颞叶明显未受累。因此我们认为它们可能代表一种迄今未被认识的新疾病实体。
