Neuro-ophthalmologic findings in konzo, an upper motor neuron disorder in Africa
Neuro-ophthalmologic findings in konzo, an upper motor neuron disorder in Africa
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DOI：
10.1177/112067210301300409
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发表时间：
2003-05-01
影响因子：
1.7
通讯作者：
Tylleskär, T
中科院分区：
文献类型：
--
作者：
Mwanza, JCK;Tshala-Katumbay, D;Tylleskär, T
目的。旨在研究 konzo 的神经眼科表现，这是一种非进行性对称性痉挛性截瘫/四肢轻瘫，与食用未充分加工的苦木薯根和低蛋白质摄入有关。方法。 21 名刚果 konzo 患者接受了神经眼科检查，包括视力测试、瞳孔光反射评估、眼球运动和偏斜评估、直接检眼镜检查和视野视野检查。还进行了客观验光，包括检影检查和角膜曲率测量，以及裂隙灯生物显微镜检查。 结果。 5 名患者有视力障碍，14 名患者视盘颞部苍白。十四人出现视野缺损，最常见的是同心收缩和周边缺损。总体而言，11 名受试者的症状符合视神经病变的诊断标准。两人在主要凝视位置出现自发性摆动性眼球震颤。轻度、中度和重度 konzo 患者均发现视野缺损和视盘苍白。 konzo 运动障碍的严重程度与视野丧失的程度之间没有发现相关性。结论。 Konzo 与视神经病变有关，少数患者有眼球震颤。尽管这种视神经病变的发病机制仍有待阐明，但受累的对称性表明其有毒性来源。我们认为氰化物会导致 konzo 神经眼科损伤。然而，konzo 患者的视神经病变与坦桑尼亚、古巴或尼日利亚流行性视神经病变、莱伯遗传性视神经病变、烟草弱视或维生素 B 缺乏症的特征并不相似。
PURPOSE. To investigate the neuro-ophthalmological manifestations in konzo, a non-progressive symmetric spastic para/tetraparesis of acute onset associated with consumption of insufficiently processed bitter cassava roots combined with a low protein intake.METHODS. Twenty-one Congolese konzo patients underwent neuro-ophthalmological investigations including visual acuity testing, assessment of light pupillary reflexes, evaluation of ocular motility and deviation, direct ophthalmoscopy, and visual field perimetry. Objective refraction including retinoscopy and keratometry, and slit-lamp biomicroscopy were also done.RESULTS. Five patients had visual impairment, and 14 had temporal pallor of the optic disc. Fourteen presented visual field defects, the most frequent being concentric constriction and peripheral defects. Overall, 11 subjects had symptoms qualifying for the diagnosis of optic neuropathy. Two had spontaneous pendular nystagmus in primary position of gaze. Visual field defects and pallor of the optic discs were found in mild, moderate and severe forms of konzo. No correlation was found between the severity of the motor disability of konzo and the extent of visual field loss.CONCLUSIONS. Konzo was associated with optic neuropathy and a few patients had nystagmus. Although the etiopathogenesis of this optic neuropathy remains to be elucidated, the symmetry of the involvement suggests a toxic origin. We suggest that cyanide causes the neuro-ophthalmological damage in konzo. However, the optic neuropathy in konzo patients does not resemble the features of the epidemic optic neuropathy in Tanzania, Cuba or Nigeria, Leber's hereditary optic neuropathy, tobacco amblyopia or vitamin B deficiency.