Lannuzel Annie, Ruberg Merle, Michel Patrick P
INSERM, UMR_S 679, Hôpital de la Salpêtrière,, Paris, France.
Mov Disord. 2008 Nov 15;23(15):2122-8. doi: 10.1002/mds.22300.
On the French West Indian island of Guadeloupe, atypical parkinsonian patients represent two-thirds of all cases of parkinsonism, which is exceptionally frequent compared to epidemiological data from European countries where atypical parkinsonism accounts for only approximately 5% of all cases. The clinical entity was a unique combination of levodopa-resistant parkinsonism, tremor, myoclonus, hallucinations, REM sleep behavior disorder and fronto-subcortical dementia. Based on the presence or the absence of supranuclear gaze palsy, two subgroups of patients were distinguished. In patients with oculomotor signs that came to autopsy, neuronal loss was found to predominate in the substantia nigra and the striatum but other brain areas were also affected, including the frontal cortex. In addition, tau-containing lesions were detected throughout the brain. Epidemiological data suggested a close association of the disease with the regular consumption of soursop, a tropical annonaceous plant. Experimental studies performed in midbrain cell cultures identified annonacin, a selective mitochondrial complex I inhibitor contained in the fruit and leaves of soursop, as a probable etiological factor. Consistent with this view, chronic administration of annonacin to rats through Alzet osmotic minipumps showed that annonacin was able to reproduce the brain lesions characteristic of the human disease.
在法属西印度群岛的瓜德罗普岛上，非典型帕金森病患者占帕金森病所有病例的三分之二，与欧洲国家的流行病学数据相比，这一比例异常之高，在欧洲国家非典型帕金森病仅占所有病例的约5%。该临床病症是左旋多巴抵抗性帕金森病、震颤、肌阵挛、幻觉、快速眼动睡眠行为障碍和额颞叶皮质下痴呆的独特组合。根据是否存在核上性凝视麻痹，区分出两个患者亚组。在有动眼神经体征且进行尸检的患者中，发现黑质和纹状体中神经元丢失占主导，但其他脑区也受到影响，包括额叶皮质。此外，在全脑检测到含tau蛋白的病变。流行病学数据表明该疾病与经常食用刺果番荔枝（一种热带番荔枝科植物）密切相关。在中脑细胞培养物中进行的实验研究确定，刺果番荔枝果实和叶子中含有的一种选择性线粒体复合物I抑制剂番荔枝素是可能的病因。与此观点一致，通过Alzet渗透微型泵对大鼠长期给药番荔枝素表明，番荔枝素能够重现人类疾病特有的脑部病变。
