AMYOTROPHIC LATERAL SCLEROSIS AND PARKINSONISM-DEMENTIA COMPLEX ON GUAM - FURTHER PATHOLOGIC STUDIES
AMYOTROPHIC LATERAL SCLEROSIS AND PARKINSONISM-DEMENTIA COMPLEX ON GUAM - FURTHER PATHOLOGIC STUDIES
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DOI：
10.1001/archneur.1966.00470130039004
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发表时间：
1966-01-01
影响因子：
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通讯作者：
KURLAND, LT
中科院分区：
文献类型：
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作者：
HIRANO, A;MALAMUD, N;KURLAND, LT
报告了来自马里亚纳群岛的 111 例病例的神经病理学研究：35 例为肌萎缩侧索硬化症 (ALS)，47 例为帕金森病-痴呆综合征 (PD) 或帕金森病； 29 名既没有 ALS 也没有 PD 的临床诊断者（这些作为比较的对照）。除了经典 ALS 的病理学特征外，神经原纤维变化在关岛的所有查莫罗 ALS 病例中也广泛分布。在关岛的所有 ALS 病例中，黑质均受到影响，其程度从宏观萎缩到微观发现色素神经元中分散的神经原纤维变化。关岛查莫罗帕金森病病例的主要神经病理学特征与之前报道的基本相似，包括特定部位的退行性改变和广泛的神经原纤维病变。对这些 PD 病例的脊髓研究表明，大约一半的病例出现了与 ALS 相似的变化。此外，在存在保存完好的神经元群的情况下，经常在脊髓中观察到节内纤维变化。 29 名查莫罗成人“对照”中的 5 名组织表现出轻微但明确的 ALS-PD 综合征常见的组织学特征；其余24例没有表现出这些具体变化。讨论了对照对象中可能的选择性特征。关岛病例中的这些观察结果进一步支持了先前提出的假设，即 ALS 和 PD 作为单一疾病实体（ALS-PD）的谱系成员相关。根据神经病理学发现讨论该综合征与其他疾病的关系。神经原纤维变化的发病机制被认为是 ALS-PD 的可能病因。
Neuropathological studies are reported in 111 cases from the Mariana Islands: 35 of amyotrophic lateral sclerosis (ALS), 47 of parkinsonism-dementia complex (PD) or parkinsonism; and 29 in whom neither ALS nor PD was clinically diagnosed (these served as controls for comparison). In addition to the pathology of classical ALS, neurofibrillary changes were widely distributed in all the Chamorro ALS cases from Guam. The substantia nigra was affected in all the ALS cases from Guam, in degrees varying from macroscopic atrophy to microscopic findings of scattered neurofibrillary changes in the pigmented neurons. The principal neuro-pathologic features in the Chamorro PD cases of Guam were essentially similar to those previously reported and consisted of specific localization of the degenerative changes and of widespread neurofibrillary lesions. Study of the spinal cord in these PD cases revealed that changes similar to those of ALS were found in approximately half of the cases. Furthermore, Intragangllonlc fibrillary changes were often observed in the spinal cord in the presence of a well-preserved neuronal population. Tissues of 5 of the 29 Chamorro adult "controls" demonstrated mild but definite histologic features common to the ALS-PD syndrome; the remaining 24 cases did not show these specific changes. The possible selective features in the control subjects are discussed. These observations in the Guam cases further support the previously proposed hypothesis that ALS and PD are related as members of a spectrum of a single disease entity: ALS-PD. The relationship of this syndrome to other diseases is discussed on the basis of neuropathological findings. The pathogenesls of neurofibrillary changes is considered as is the possible etiology of ALS-PD.