法国北部进行性核上性麻痹的地理聚集区。
A geographical cluster of progressive supranuclear palsy in northern France.
作者信息
Caparros-Lefebvre Dominique, Golbe Lawrence I, Deramecourt Vincent, Maurage Claude-Alain, Huin Vincent, Buée-Scherrer Valerie, Obriot Helene, Sablonnière Bernard, Caparros Francois, Buée Luc, Lees Andrew J
机构信息
From the Unit of Neurology (D.C.-L.), Centre Hospitalier de Wattrelos, France; Department of Neurology (L.I.G.), Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ; University of Lille Nord de France (V.D., C.-A.M., V.H., V.B.-S., H.O., B.S., F.C., L.B.), INSERM UMR 1172, Batiment JPARC, France; and Reta Lila Weston Institute for Neurological Studies (A.J.L.), London, UK.
出版信息
Neurology. 2015 Oct 13;85(15):1293-300. doi: 10.1212/WNL.0000000000001997. Epub 2015 Sep 9.
OBJECTIVE
To describe a cluster of progressive supranuclear palsy (PSP) in northern France. PSP has not been reported in geographical, temporal, or occupational clusters. A unit of Neurology and Neurogeriatrics opened in 2005 at the Centre Hospitalier de Wattrelos, serving the population of Wattrelos and Leers (combined population 51,551) and parts of neighboring towns. For most of the 20th century, this area was a center for chromate and phosphate ore processing, textile dyeing, and tanning. Significant industrial waste persists close to residential areas.
METHODS
From 2005 to 2014, 92 patients with PSP at Centre Hospitalier de Wattrelos were identified and studied. Detailed residential data were available in the medical records. Eighty cases have had magnetic resonance head scanning and 60 have died, of whom 13 have been examined neuropathologically.
RESULTS
The ratio of observed to expected PSP incidence over the period 2005 to 2012 was 12.3 (95% confidence interval: 7.4-35.9). Mean onset age was 74.3 years. The Richardson syndrome/PSP-parkinsonism ratio was 43%/42%. Four other phenotypes each occurred in 2% to 5%. Onset was gait/balance difficulty in 52%. None of the 92 affected patients were relatives and 7 were of North African ancestry. MRI was compatible with a clinical diagnostic of PSP in all cases. Histopathologic examination confirmed neurofibrillary degeneration and tufted astrocytes in all autopsied cases. Western blots revealed a typical tau 4R doublet. The tau H1 haplotype occurred in 95.8% of cases' chromosomes.
CONCLUSIONS
We have identified a cluster of PSP in a geographical area with severe environmental contamination by industrial metals.
目的
描述法国北部的一组进行性核上性麻痹（PSP）病例。此前未见PSP在地理、时间或职业群体中聚集发病的报道。瓦特勒洛斯中心医院于2005年开设了神经内科和神经老年病科，服务于瓦特勒洛斯和勒尔的居民（总人口51,551）以及邻近城镇的部分地区。在20世纪的大部分时间里，该地区是铬酸盐和磷酸盐矿石加工、纺织品染色及制革的中心。大量工业废料仍留存于居民区附近。
方法
2005年至2014年期间，在瓦特勒洛斯中心医院确诊并研究了92例PSP患者。病历中有详细的居住数据。80例患者进行了头部磁共振扫描，60例患者死亡，其中13例进行了神经病理学检查。
结果
2005年至2012年期间观察到的PSP发病率与预期发病率之比为12.3（95%置信区间：7.4 - 35.9）。平均发病年龄为74.3岁。理查森综合征/PSP帕金森综合征的比例为43%/42%。其他四种表型各占2%至5%。52%的患者起病表现为步态/平衡困难。92例受累患者均无亲属关系，7例为北非血统。所有病例的MRI结果均与PSP的临床诊断相符。组织病理学检查在所有尸检病例中均证实有神经原纤维变性和簇状星形胶质细胞。蛋白质免疫印迹显示典型的tau 4R双峰。tau H1单倍型出现在95.8%的病例染色体中。
结论
我们在一个受到工业金属严重环境污染的地理区域发现了一组PSP病例。
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