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惰性抗Hu相关副肿瘤性感觉神经病

Indolent anti-Hu-associated paraneoplastic sensory neuropathy.

作者信息

Graus F, Bonaventura I, Uchuya M, Valls-Solé J, Reñé R, Leger J M, Tolosa E, Delattre J Y

机构信息

Service of Neurology, Hospital Clinic i Provincial, Barcelona, Spain.

出版信息

Neurology. 1994 Dec;44(12):2258-61. doi: 10.1212/wnl.44.12.2258.

Abstract

Paraneoplastic sensory neuropathy (PSN) usually runs a subacute progressive course, leaving the patient with severe sensory dysfunction in weeks to months. We describe five patients with PSN, high titers of anti-Hu antibodies (type 1 antineuronal nuclear autoantibodies), and an indolent clinical course. The patients had a median age of 55 years (range, 41 to 72). Four had small-cell (3) or undifferentiated large-cell (1) lung cancer. Patients presented with mild, asymmetric sensory symptoms; in two, the neuropathy was predominant in the arms. Two patients also had a visceral neuropathy causing gastrointestinal dysfunction. The PSN was stable or progressed very slowly without treatment for a median of 18 months (range, 5 to 32) and remained so after treatment with immunoglobulins (1 patient), chemotherapy (3), or both therapies (1). All patients were ambulatory, leading an independent life up until the time of the last visit or until death from the tumor (2 patients). The median follow-up was 36 months (range, 22 to 52). A paraneoplastic origin should be considered in patients with mild, very slowly progressive sensory neuropathies.

摘要

副肿瘤性感觉神经病(PSN)通常呈亚急性进行性病程,在数周数月内使患者出现严重的感觉功能障碍。我们描述了5例患有PSN、抗Hu抗体(1型抗神经元核自身抗体)滴度高且临床病程进展缓慢的患者。这些患者的中位年龄为55岁(范围41至72岁)。4例患有小细胞肺癌(3例)或未分化大细胞肺癌(1例)。患者表现为轻度、不对称的感觉症状;其中2例患者的神经病变以手臂为主。2例患者还存在导致胃肠功能障碍的内脏神经病变。PSN在未治疗的情况下稳定或进展非常缓慢,中位时间为18个月(范围5至32个月),在接受免疫球蛋白治疗(1例)、化疗(3例)或两种治疗(1例)后仍保持如此。所有患者在最后一次就诊时或直至死于肿瘤(2例)之前均能行走并独立生活。中位随访时间为36个月(范围22至52个月)。对于轻度、进展非常缓慢的感觉神经病患者,应考虑副肿瘤性病因。

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