Department of Neurology, Mayo Clinic, Rochester, MN, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905,USA.
Lancet Neurol. 2010 Jan;9(1):55-66. doi: 10.1016/S1474-4422(09)70296-0. Epub 2009 Nov 27.
Between November, 2006, and May, 2008, a subacute neurological syndrome affected workers from two swine abattoirs in Minnesota and Indiana who had occupational exposure to aerosolised porcine brain. We aimed to describe the pathogenic and immunological characteristics of this illness.
All patients from two abattoirs who presented or were referred to the Mayo Clinic (Rochester, MN, USA) with neurological symptoms were included. We recorded details of exposure to aerosolised brain tissue and did comprehensive neurological, laboratory, neuroimaging, electrophysiological, pathological, and autoimmune serological assessments. Healthy controls were recruited from the community and from workers at the plant in Minnesota.
24 patients were identified (21 from Minnesota, three from Indiana). The shortest duration from first exposure to symptom onset was 4 weeks. No infectious agent that could trigger disease was identified. All patients developed polyradiculoneuropathy, which was usually sensory predominant and painful. Two patients had initial CNS manifestations: transverse myelitis and meningoencephalitis. Nerve conduction studies localised abnormalities to the most proximal and distal nerve segments. Quantitative sensory and autonomic testing revealed involvement of large and small sensory fibres and sweat fibres. MRI showed prominent abnormalities of roots and ganglia. Nerve biopsies identified mild demyelination, axonal degeneration, and perivascular inflammation. Protein concentrations were high in the CSF of 18 (86%) of 21 patients. Sera from all patients and 29 (34%) of 85 unaffected workplace controls (but none of 178 community controls) had a distinctive neural-reactive IgG; 75% of patients' sera contained an IgG specific to myelin basic protein. Seropositivity correlated directly with exposure risk in patients and controls. 17 patients required immunomodulatory therapies, six improved spontaneously, and one was lost to follow-up after exposure stopped.
The neurological disorder described is autoimmune in origin and is related to occupational exposure to multiple aerosolised porcine brain tissue antigens. The pattern of nerve involvement suggests vulnerability of nerve roots and terminals where the blood-nerve barrier is most permeable.
Mayo Clinic Foundation; Minnesota Department of Health; Centers for Disease Control and Prevention.
2006 年 11 月至 2008 年 5 月期间，明尼苏达州和印第安纳州的两家生猪屠宰场的工人因职业性接触气溶胶化猪脑而出现亚急性神经综合征。我们旨在描述这种疾病的发病机制和免疫学特征。
两家屠宰场的所有出现神经症状或被转介至美国明尼苏达州罗切斯特市梅奥诊所的患者均被纳入研究。我们记录了暴露于气溶胶化脑组织的详细情况，并进行了全面的神经学、实验室、神经影像学、电生理学、病理学和自身免疫血清学评估。健康对照者从社区和明尼苏达州工厂的工人中招募。
共确定了 24 例患者（21 例来自明尼苏达州，3 例来自印第安纳州）。从首次接触到症状出现的最短时间为 4 周。未发现任何可引发疾病的感染性病原体。所有患者均出现多发性神经根神经病，通常以感觉为主且疼痛。2 例患者最初出现中枢神经系统表现：横断性脊髓炎和脑膜脑炎。神经传导研究将异常定位在最近端和最远端的神经节段。定量感觉和自主神经检测显示大、小感觉纤维和汗腺纤维受累。MRI 显示神经根和神经节明显异常。神经活检发现轻度脱髓鞘、轴突变性和血管周围炎症。21 例患者中的 18 例（86%）CSF 蛋白浓度升高。所有患者的血清和 85 例未受影响的工作场所对照者（但 178 例社区对照者无一例）的血清中均存在一种独特的神经反应性 IgG；75%的患者血清中含有一种针对髓鞘碱性蛋白的 IgG。血清阳性与患者和对照者的暴露风险直接相关。17 例患者需要免疫调节治疗，6 例自发改善，1 例在停止暴露后失访。
所描述的神经疾病是自身免疫性的，与职业性接触多种气溶胶化猪脑组织抗原有关。神经受累的模式提示神经根和神经末梢最易渗透的部位易受影响，这些部位的血-神经屏障最易渗透。
梅奥诊所基金会；明尼苏达州卫生部；疾病控制与预防中心。
