Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal autoantibodies

被引:143
作者
Uchuya, M
Graus, F
Vega, F
Rene, R
Delattre, JY
机构
[1] HOP LA PITIE SALPETRIERE,DEPT NEUROL,NEUROIMMUNOL LAB,SERV NEUROL,F-75651 PARIS 13,FRANCE
[2] HOP LA PITIE SALPETRIERE,UNITE INSERM 134,F-75651 PARIS 13,FRANCE
[3] HOSP CLIN BARCELONA,SERV NEUROL,BARCELONA,SPAIN
[4] PRINCIIPES ESPANA,LHOSPITALET LLOBR,SPAIN
关键词
immunoglobulin treatment; paraneoplastic neurological syndromes; antineuronal autoantibodies; Rankin scale;
D O I
10.1136/jnnp.60.4.388
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective-To evaluate the effect of intravenous high dose human immunoglobulin (IVIg) therapy on the clinical course and autoantibody titres of patients with neurological paraneoplastic syndromes. Methods-Twenty two patients with paraneoplastic encephalomyelitis and sensory neuronopathy syndrome associated with anti-au antibodies (18) or paraneoplastic cerebellar degeneration (PCD) with anti-Yo antibodies (four), were treated with 1-26 (mean 5.8) cycles of IVIg. The Rankin scale was used to evaluate the response. Results-The only serious toxicity was one case of haemolytic anaemia. Twenty one patients were evaluable for therapeutic response. One patient, with subacute sensory neuronopathy (SSN), improved for at least 15 months, 10 remained stable (eight with anti-Hu and two with anti-Yo antibodies), and 10 deteriorated (eight with anti-Hu and two with anti-Yo antibodies). In seven of the 10 patients who stabilised, the syndrome had already made a plateau when the treatment was started but three patients (one with anti-au and two with anti-Yo antibodies) who had still been progressing stabilised for six, eight, and more than 48 months, including one patient with SSN who achieved stabilisation when the neurological dysfunction was only moderate (Rankin scale = 3). Another patient with SSN and initial stable response worsened when Mg was reduced and improved when it was increased. No significant predictive factors of outcome could be identified but improvement or stabilisation was more frequent in patients with isolated involvement of the peripheral nervous system (62%) than in patients with evidence of CNS damage (37%) at the onset of treatment. Stabilisation in patients with CNS involvement was only achieved when the neurological dysfunction was already severe (Rankin scale > 3). The titres of autoantibodies did not change significantly. Conclusion-Treatment with IVIg at the doses given in the present protocol was not effective in paraneoplastic CNS syndromes associated with antineuronal antibodies. The role of this regime in the treatment of SSN should be further evaluated.
引用
收藏
页码:388 / 392
页数:5
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