Heiman-Patterson T D, Bird S J, Parry G J, Varga J, Shy M E, Culligan N W, Edelsohn L, Tatarian G T, Heyes M P, Garcia C A
Department of Neurology, Jefferson Medical College, Philadelphia, PA 19107-5083.
Ann Neurol. 1990 Oct;28(4):522-8. doi: 10.1002/ana.410280409.
In 1989, the Centers for Disease Control recognized the existence of an epidemic illness characterized by myalgia and eosinophilia in individuals taking preparations containing L-tryptophan. We evaluated 3 patients with eosinophilia-myalgia syndrome who presented with subacute progressive neuropathies. The neuropathies were predominantly motor and maximal in the lower extremities. Two patients were confined to a wheelchair and one was ventilator-dependent and bedridden. Sensory loss predominantly involved small fiber modalities. Electrophysiological studies showed multifocal marked conduction slowing and conduction block indicating segmental demyelination, with associated axonal degeneration that was accentuated distally. Examination of sural nerve biopsy specimens demonstrated axonal degeneration in all 3 patients and perivascular infiltrates in 2. Levels of quinolinic acid, a neurotoxic metabolite of L-tryptophan, were elevated in the cerebrospinal fluid in the 2 patients in whom it was measured. The cause of the neuropathy is unknown but may include immune mechanisms or toxicity of eosinophils, L-tryptophan, its metabolic products, or contaminants within L-tryptophan preparations.
1989年,美国疾病控制中心认识到,服用含L-色氨酸制剂的个体中存在一种以肌痛和嗜酸性粒细胞增多为特征的流行性疾病。我们评估了3例伴有嗜酸性粒细胞增多性肌痛综合征且出现亚急性进行性神经病变的患者。这些神经病变主要为运动性,且在下肢最为严重。两名患者只能坐轮椅,一名患者依赖呼吸机且卧床不起。感觉丧失主要累及小纤维感觉模式。电生理研究显示多灶性明显的传导减慢和传导阻滞,提示节段性脱髓鞘,并伴有轴索性变性,且远端更为明显。对腓肠神经活检标本的检查显示,所有3例患者均有轴索性变性,2例有血管周围浸润。在测定的2例患者的脑脊液中,L-色氨酸的神经毒性代谢产物喹啉酸水平升高。神经病变的原因尚不清楚,但可能包括免疫机制或嗜酸性粒细胞、L-色氨酸、其代谢产物或L-色氨酸制剂中的污染物的毒性。