Hof P R, Knabe R, Bovier P, Bouras C
Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, NY 10029.
Acta Neuropathol. 1991;82(4):321-6. doi: 10.1007/BF00308819.
We report the neuropathological evaluation of a 24-year-old autistic woman suffering from a residual state of infantile autism and presenting with self-injury behavior since childhood. Her behavior included head-banging, eye-gouging and self-biting. All intended therapeutic measures remained without effect, including high doses of psychotropic drugs. At autopsy, numerous neurofibrillary tangles were found in the perirhinal and entorhinal cortex where they were frequently grouped in nests or clusters. A few neurofibrillary tangles were also observed in the amygdala and in the prepiriform and orbito-frontal cortex. In the cortex, tangles were located in both layers II and III. There were no neuritic plaques or amyloid deposits. Interestingly, neurofibrillary tangles have been described in brains of individuals who had experienced repeated head injuries such as boxers (dementia pugilistica) and soccer players, suggesting that in our case a similar mechanism induced tangle formation and resulted in the loss of selective neuronal populations.
我们报告了一名24岁自闭症女性的神经病理学评估情况。该女性患有婴儿自闭症残留状态,自童年起就有自我伤害行为。她的行为包括撞头、抠眼睛和咬自己。所有预期的治疗措施均无效,包括高剂量的精神药物。尸检时,在嗅周皮质和内嗅皮质发现了大量神经原纤维缠结,它们常聚集成巢状或簇状。在杏仁核、梨状前皮质和眶额皮质也观察到少量神经原纤维缠结。在皮质中,缠结位于第II层和第III层。没有神经炎性斑块或淀粉样沉积。有趣的是,在经历过反复头部损伤的个体(如拳击手,患拳击员痴呆症;足球运动员)的大脑中也描述过神经原纤维缠结,这表明在我们的病例中,类似的机制诱导了缠结的形成,并导致了选择性神经元群体的丧失。