Etiology of Retinal and Cerebellar Pathology in Western Pacific Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex.

IF 3.1 Q1 OPHTHALMOLOGY Eye and Brain Pub Date : 2020-07-16 eCollection Date: 2020-01-01 DOI:10.2147/EB.S260823

Peter S Spencer

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引用次数: 11

Abstract

Purpose: To reexamine the etiology of a unique retinal pathology (linear and vermiform sub-retinal tubular structures) described among subjects with and without neurodegenerative disease in former high-incidence foci of Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in Guam (USA) and the Kii peninsula of Honshu island (Japan).

Methods: Analysis of published and unpublished reports of 1) ALS/PDC and the retinal and cerebellar pathology associated therewith and 2) exogenous neurotoxic factors associated with ALS/PDC and the developing retina and cerebellum.

Results: ALS/PDC retinal and cerebellar pathology matches persistent retinal and cerebellar dysplasia found in laboratory animals given single in utero or postnatal systemic treatment with cycasin, the principal neurotoxic component in the seed of cycad plants traditionally used for food (Guam) or oral medicine (Kii-Japan), both of which have been linked to the human neurodegenerative disease.

Conclusion: ALS/PDC-associated retinal and cerebellar dysplasia could arise from in utero exposure to methylazoxymethanol, the genotoxic metabolite of cycasin that results from maternal ingestion of this azoxyglucoside. These results support the environmental toxic etiology of retinal and brain pathology in ALS/PDC.

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西太平洋肌萎缩性侧索硬化症和帕金森-痴呆综合症视网膜和小脑病理的病因学。

目的:重新检查一种独特的视网膜病理(线状和蚓状视网膜下管状结构)的病因，在美国关岛和日本本州岛Kii半岛的西太平洋肌萎缩性侧索硬化症和帕金森-痴呆复发症(ALS/PDC)的前高发灶中，有和没有神经退行性疾病的受试者中描述。方法:对已发表和未发表的ALS/PDC及其相关视网膜和小脑病理报告和与ALS/PDC及发育中的视网膜和小脑相关的外源性神经毒性因子进行分析。结果:ALS/PDC视网膜和小脑病理与在实验室动物中发现的持续性视网膜和小脑发育不良相吻合，实验动物在子宫内或出生后接受单一的苏铁素系统治疗，苏铁素是苏铁植物种子中的主要神经毒性成分，传统上用于食物(关岛)或口服药物(Kii-Japan)，这两种植物都与人类神经退行性疾病有关。结论:ALS/ pdc相关的视网膜和小脑发育不良可能由子宫内暴露于甲基甲氧基甲醇引起，甲基甲氧基甲醇是苏铁素的遗传毒性代谢物，由母体摄入这种偶氮氧基糖苷引起。这些结果支持ALS/PDC患者视网膜和脑病理的环境毒性病因学。

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来源期刊

Eye and Brain OPHTHALMOLOGY-

CiteScore

7.90

自引率

2.30%

发文量

审稿时长

16 weeks

期刊介绍： Eye and Brain is an international, peer-reviewed, open access journal focusing on basic research, clinical findings, and expert reviews in the field of visual science and neuro-ophthalmology. The journal’s unique focus is the link between two well-known visual centres, the eye and the brain, with an emphasis on the importance of such connections. All aspects of clinical and especially basic research on the visual system are addressed within the journal as well as significant future directions in vision research and therapeutic measures. This unique journal focuses on neurological aspects of vision – both physiological and pathological. The scope of the journal spans from the cornea to the associational visual cortex and all the visual centers in between. Topics range from basic biological mechanisms to therapeutic treatment, from simple organisms to humans, and utilizing techniques from molecular biology to behavior. The journal especially welcomes primary research articles or review papers that make the connection between the eye and the brain. Specific areas covered in the journal include: Physiology and pathophysiology of visual centers, Eye movement disorders and strabismus, Cellular, biochemical, and molecular features of the visual system, Structural and functional organization of the eye and of the visual cortex, Metabolic demands of the visual system, Diseases and disorders with neuro-ophthalmic manifestations, Clinical and experimental neuro-ophthalmology and visual system pathologies, Epidemiological studies.