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Objectives <p>To identify factors associated with poor response to initial first-line immunotherapy in pediatric patients with anti-NMDAR encephalitis.</p> Methods <p>This monocentric prospective cohort study included pediatric anti-NMDAR encephalitis between January 2017 and December 2021. The modified Rankin Scale (mRS) score was used to assess neurological severity.</p> Results <p>This study included 152 patients, 74 of them (48.7%) had a poor response to initial first-line immunotherapy. Decreased consciousness (<i>p</i> = 0.001, OR = 6.889), autonomic dysfunction/central hypoventilation (<i>p</i> = 0.003, OR = 4.704), speech dysfunction (<i>p</i> = 0.007, OR = 4.272), mRS score before immunotherapy &gt; = 4 points (<i>p</i> &lt; 0.001, OR = 10.968), and age &lt; = 3 years (<i>p</i> = 0.044, OR = 5.169) significantly affected the response to initial first-line immunotherapy. At 12 months, the good responders demonstrated a significantly better outcome than did the poor responders (100% vs. 74.3%, <i>p</i> &lt; 0.001), although the recurrence rate was comparable between the two group (11.5% vs. 14.9%, <i>p</i> = 0.544). Of the 74 poor responders, 45 patients underwent subsequent immunotherapy, and they exhibited a greater proportion of favorable outcomes compared to the group without subsequent immunotherapy at 12 months.</p> Conclusion <p>Pediatric anti-NMDAR encephalitis with decreased consciousness, autonomic dysfunction/central hypoventilation, speech dysfunction, mRS score &gt; = 4 points before immunotherapy, and age &lt; = 3 years had a greater risk of poor response to initial first-line immunotherapy, and poorer prognosis. Subsequent immunotherapy can improve long-term prognosis for poor responders.</p>

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Risk factors for poor response to initial first-line immunotherapy and subsequent immunotherapy on prognosis in pediatric anti-NMDA receptor encephalitis

  • 期刊论文

  • 发表日期：

    2025年5月16日

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  • Yuhang Li,
  • Hanyu Luo,
  • Zhiwei Yu,
  • Jiaxin Yang,
  • Yan Jiang,
  • Xiujuan Li,
  • Jiannan Ma,
  • Li Jiang

摘要

Objectives

To identify factors associated with poor response to initial first-line immunotherapy in pediatric patients with anti-NMDAR encephalitis.

Methods

This monocentric prospective cohort study included pediatric anti-NMDAR encephalitis between January 2017 and December 2021. The modified Rankin Scale (mRS) score was used to assess neurological severity.

Results

This study included 152 patients, 74 of them (48.7%) had a poor response to initial first-line immunotherapy. Decreased consciousness (p = 0.001, OR = 6.889), autonomic dysfunction/central hypoventilation (p = 0.003, OR = 4.704), speech dysfunction (p = 0.007, OR = 4.272), mRS score before immunotherapy > = 4 points (p < 0.001, OR = 10.968), and age < = 3 years (p = 0.044, OR = 5.169) significantly affected the response to initial first-line immunotherapy. At 12 months, the good responders demonstrated a significantly better outcome than did the poor responders (100% vs. 74.3%, p < 0.001), although the recurrence rate was comparable between the two group (11.5% vs. 14.9%, p = 0.544). Of the 74 poor responders, 45 patients underwent subsequent immunotherapy, and they exhibited a greater proportion of favorable outcomes compared to the group without subsequent immunotherapy at 12 months.

Conclusion

Pediatric anti-NMDAR encephalitis with decreased consciousness, autonomic dysfunction/central hypoventilation, speech dysfunction, mRS score > = 4 points before immunotherapy, and age < = 3 years had a greater risk of poor response to initial first-line immunotherapy, and poorer prognosis. Subsequent immunotherapy can improve long-term prognosis for poor responders.