Komachi H, Okeda R, Ishii N, Yanagisawa K, Yamada M, Miyatake T
Department of Neurology, Tokyo Medical and Dental University, Japan.
J Neurol. 1994 Oct;241(10):592-6. doi: 10.1007/BF00920622.
Ophthalmoplegia and dementia are not usually observed in patients with amyotrophic lateral sclerosis. We report the case of a 60-year-old female with ophthalmoplegia and frontal-type dementia which appeared at an early stage of her illness that presented with dysarthria and weakness in the upper extremities. Notable autopsy findings in the central nervous system were, in addition to the degeneration of upper and lower motor neurons, moderate neuronal loss and spongy degeneration in layer II of the frontal cortex with prominent astrocytosis, and moderate neuronal loss with astrocytosis in both the substantia nigra and the red nucleus. Central chromatolysis of a few neurons in the oculomotor nucleus was seen. This case is considered to be a new subtype of motor neuron disease.
肌萎缩侧索硬化症患者通常不会出现眼肌麻痹和痴呆症状。我们报告了一例60岁女性患者，她在疾病早期出现了眼肌麻痹和额叶型痴呆，同时伴有构音障碍和上肢无力。中枢神经系统显著的尸检结果除了上下运动神经元变性外，额叶皮质II层有中度神经元丢失和海绵状变性，伴有明显的星形细胞增生，黑质和红核也有中度神经元丢失和星形细胞增生。动眼神经核中可见少数神经元中央性染色质溶解。该病例被认为是运动神经元病的一种新亚型。
