@article{oai:niigata-u.repo.nii.ac.jp:00024382, author = {若林, 允甫 and 小柳, 新策}, issue = {12}, journal = {新潟医学会雑誌, 新潟医学会雑誌}, month = {Dec}, note = {Here, we present two autopsy cases, first of which was clinically similar to Machado-Joseph disease, and the second one, her neuropathological findings were almost the same to the first case. In the first case, her disease began as Marie's ataxia at age 17. Then, extrapyramidal signs, external ophthalmoplegia, symptome of secondary motor neuron impairment and autonomic nerve disturbance developed gradually. She died of pneumonia at age 35. In the second case, her unsteady gait began early in her life and became getting worse. Then, superficial and deep sensory impairment, secondary motor nerve impairment, and external ophthalmoplegia developed gragually. She died at age 69. Neuropathological findings of these two cases were almost the same, which were summarized as below: 1)Subthalamo-pallidal and Pallido-nigral system, 2)Dentato-rubral system, 3)Ponto-cerebellar system, 4)Cranial nerve nuclei, 5)Spinal cord(spino-cerebellar tract, posterior funiculus, lateral pyramidal tract, posterior funiculus, lateral pyramidal tract, anterior horn, Clarch's column, and only in the first case, intermedio-lateral column). With regard to the distribution of the degenerative lesion, the present cases have a great similarity to Friedreich's ataxia, but the lesions outside spinal cord are far severe. So, we can not classify the present cases into any of known disorders, and expect these types of spino-cerebellar degeneration to be estabrished as a unique system degenerative disease of the central nervous system.}, pages = {721--725}, title = {3) ジョセフ病型を示した脊髄小脳変性症の2剖検例(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)}, volume = {100}, year = {1986} }