Román G C
Neuroepidemiology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
J Neurol Sci. 1994 Dec 1;127(1):11-28. doi: 10.1016/0022-510x(94)90130-9.
An epidemic outbreak of peripheral neuropathy affected Cuba in 1992-93 resulting in 50,862 cases (national cumulative incidence rate (CIR) 461.4 per 100,000). Clinical forms included retrobulbar optic neuropathy, sensory and dysautonomic peripheral neuropathy, dorsolateral myeloneuropathy, sensorineural deafness, dysphonia and dysphagia, spastic paraparesis, and mixed forms. For epidemiological purposes, cases were classified as optic forms (CIR 242.39) or peripheral forms (CIR 219.25). Increased risk was found among smokers (odds ratio (OR) 4.9), those with history of missing meals (OR 4.7) resulting in lower intake of animal protein, fat, and foods that contain B-vitamins, combined drinking and smoking (OR 3.5), weight loss (OR 2.8), excessive sugar consumption (OR 2.7) and heavy drinking (OR 2.3). Optic neuropathy was characterized by decreased vision, bilateral and symmetric central or cecocentral scotomata, and loss of color vision due to selective lesion of the maculopapillary bundles. Peripheral neuropathy was a distal axonopathy lesion affecting predominantly large myelinated axons. Deafness produced selective high frequency (4-8 kHz) hearing loss. Myelopathy lesions combined dorsal column deficits and pyramidal involvement of lower limbs with spastic bladder. Clinical features were those of Strachan syndrome and beriberi. Intensive search for neurotoxic agents, in particular organophosphorus esters, chronic cyanide, and trichloroethylene intoxication, yielded negative results. Treatment of patients with B-group vitamins and folate produced rewarding results. Most patients improved significantly and less than 0.1% of them remained with sequelae; there were no fatal cases. Supplementation of multivitamins to the entire Cuban population resulted in curbing of the epidemic. Overt malnutrition was not present, but a deficit of micronutrients, in particular thiamine, cobalamine, folate and sulfur amino acids appears to have been a primary determinant of this epidemic.
1992年至1993年，一场周围神经病变的流行病爆发，侵袭了古巴，导致50862例病例（全国累计发病率为每10万人461.4例）。临床症状包括球后视神经炎、感觉性和自主神经功能障碍性周围神经病变、脊髓背外侧神经病变、感音神经性耳聋、发音困难和吞咽困难、痉挛性截瘫以及混合型。出于流行病学目的，病例被分为视神经型（发病率242.39）或周围神经型（发病率219.25）。研究发现，吸烟者（比值比（OR）为4.9）、有不规律饮食史（OR为4.7）导致动物蛋白、脂肪和含B族维生素食物摄入量较低者、同时饮酒和吸烟者（OR为3.5）、体重减轻者（OR为2.8）、过量食用糖者（OR为2.7）以及大量饮酒者（OR为2.3）患病风险增加。视神经病变的特征为视力下降、双侧对称的中心或中心暗点，以及由于黄斑乳头束选择性损伤导致的色觉丧失。周围神经病变是一种主要影响大的有髓轴突的远端轴索性病变。耳聋导致选择性高频（4 - 8千赫）听力损失。脊髓病损合并了背柱功能缺陷以及下肢锥体受累和痉挛性膀胱。临床特征符合斯特罗恩综合征和脚气病。对神经毒剂，特别是有机磷酸酯、慢性氰化物和三氯乙烯中毒进行的深入排查结果为阴性。用B族维生素和叶酸治疗患者取得了良好效果。大多数患者显著改善，不到0.1%的患者留有后遗症；无死亡病例。对全体古巴人口补充多种维生素后疫情得到了控制。虽然没有明显的营养不良，但微量营养素缺乏，特别是硫胺素、钴胺素、叶酸和含硫氨基酸缺乏似乎是此次疫情的主要决定因素。
