Marés-Segura R
Sección de Neurología, Hospital Universitari Joan XXIII, Tarragona, España.
Rev Neurol. 2000;31(12):1219-23.
We reviewed the bibliography of various paraneoplastic syndromes which may show spinal involvement.
Paraneoplastic sensory neuropathy, myelitis and rigidity syndromes may present alone or as part of a generalized syndrome of paraneoplastic encephalomyelitis/sensory neuronopathy, usually associated with small cell cancer of the lung and anti-Hu antibodies. Amyotrophic lateral sclerosis and subacute necrotizing myelopathy of paraneoplastic origin are very rare, although casual association of these conditions with cancer cannot be ruled out. Subacute motor neuronopathy is linked to lymphoproliferative syndromes and breast cancer has been reported associated with cases of primary lateral sclerosis and the stiff man syndrome.
Various conditions of paraneoplastic origin may affect the spinal cord. Neurological symptoms may precede the diagnosis of neoplasia, are serious and do not usually respond to either immunosuppressive treatment or treatment of the underlying neoplasm, although there are exceptions. The presence of specific antibodies in some cases facilitates early diagnosis and shows the importance of immune mechanisms in these diseases. The paraneoplastic motor neuron syndromes may present atypically: at early or late age, be of slow evolution and associated with raised protein levels of the cerebrospinal fluid or with paraproteinemia.
我们回顾了各种可能出现脊髓受累的副肿瘤综合征的文献。
副肿瘤性感觉神经病、脊髓炎和僵人综合征可能单独出现，或作为副肿瘤性脑脊髓炎/感觉神经元病综合征的一部分出现，通常与肺癌和抗Hu抗体相关。副肿瘤性起源的肌萎缩侧索硬化症和亚急性坏死性脊髓病非常罕见，尽管不能排除这些疾病与癌症的偶然关联。亚急性运动神经元病与淋巴增殖性综合征有关，乳腺癌与原发性侧索硬化症和僵人综合征的病例有关。
各种副肿瘤性起源的疾病可能影响脊髓。神经症状可能在肿瘤诊断之前出现，症状严重，通常对免疫抑制治疗或潜在肿瘤的治疗均无反应，尽管也有例外情况。某些病例中特异性抗体的存在有助于早期诊断，并显示了免疫机制在这些疾病中的重要性。副肿瘤性运动神经元综合征可能表现不典型：在早年或晚年出现，进展缓慢，并伴有脑脊液蛋白水平升高或副蛋白血症。
