Pathological studies on Parkinson's disease and amyotrophic lateral sclerosis

在204例连续尸检中，迷走神经背核α-突触核蛋白的病理改变与心交感神经酪氨酸羟化酶免疫反应活性降低密切相关。我们报告了各种肌萎缩侧索硬化（ALS）类型的FUS病理学，日本家族性ALS患者的临床和神经病理学发现，这些患者具有新的TDP-43 A315 E突变，存在几种外周蛋白阳性的Bunina小体，不仅在ALS中而且在许多神经退行性疾病中出现视神经磷酸酶。

In our 204 consecutive autopsied cases, there were close relationship between α-synuclein pathology in dorsal vagal nuculei and the decrease of tyrosine hydroxylase immunorectivites in cardiac sympathetic nerves. We reported FUS pathology in various amyotrophic lateral sclerosis (ALS) types, clinical and neuropathological findings of Japanese familial ALS patients with a novel TDP-43 A315E mutation, presence of several peripherin-positive Bunina bodies, and appearance of optineurin not only in ALS but also in many neurodegenerative conditions.