Nodding syndrome: origins and natural history of a longstanding epileptic disorder in sub-Saharan Africa
Nodding syndrome: origins and natural history of a longstanding epileptic disorder in sub-Saharan Africa
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DOI：
10.4314/ahs.v13i2.1
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发表时间：
2013-01-01
影响因子：
1
通讯作者：
Jilek-Aall, L.
中科院分区：
文献类型：
--
作者：
Spencer, P. S.;Palmer, V. S.;Jilek-Aall, L.
背景：20世纪60年代在坦桑尼亚的Wapogoro部落首次报道了重复的非自愿点头。目的：我们描述了Wapogoro部落中点头的自然历史，特别参考了最早报道的发病日期。方法：分析1960 ~ 1971年间150例患者的临床资料。结果：伴有或不伴有大惊厥的头点头在33/150（接近20%）的病例中出现，主要是家族性的，按性别平均分布。在1934-1962年期间，出现点头的年龄范围为2-22岁（平均：与10岁相似）。头点头早于大痉挛发作长达12个月，随着时间的推移，运动和精神运动缺陷表明脑损伤的发展。33例中有14例死亡年龄在13-39岁（平均：相似于20岁），19例16-28岁（平均：相似于16岁）仍然存活。结论：Wapogoro部落中关于点头（amesinzia kichwa，斯瓦希里语）的历史记载符合2012年8月世界卫生组织（WHO）对可能的点头综合征的病例定义。据报道，点头综合征在该人群中存在至少80年，是一种进行性癫痫发作障碍，可导致全身抽搐（kifafa）、脑损伤和死亡。
Background: Repetitive involuntary head nodding was first reported in the 1960s in the Wapogoro tribe of Tanzania.Objectives: We describe the natural history of head nodding in the Wapogoro tribe, with special reference to the earliest reported dates of onset.Methods: We analyzed clinical data from 150 historical patients seen between 1960 and 1971.Results: Head nodding with or without grand mal convulsions was present in 33/150 (similar to 20%) cases, was mostly familial and equally distributed by gender. Age at onset of head nodding ranged from 2-22 years (mean: similar to 10 years) in the period 1934-1962. Head nodding preceded onset of grand mal convulsions by up to 12 months, and motor and psychomotor deficits indicative of brain damage developed with time. Fourteen of the 33 cases died at 13-39 years of age (mean: similar to 20 years) while nineteen aged 16-28 years (mean: similar to 16 years) were still alive.Conclusion: Historical accounts of head nodding (amesinzia kichwa, Swahili) among the Wapogoro tribe fit the August 2012 World Health Organization (WHO) case definition of probable Nodding Syndrome. Reported to have existed in this population for at least 80 years, Nodding Syndrome is a progressive seizure disorder that leads to generalized convulsions (kifafa), brain damage and death.