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Clinical profile and treatment response in patients with CASPR2 antibody-associated neurological disease
Annals of Indian Academy of Neurology ( IF 1.9 ) Pub Date : 2021-03-01 , DOI: 10.4103/aian.aian_574_20 Sumanth Shivaram 1 , Madhu Nagappa 1 , Doniparthi V Seshagiri 1 , Anita Mahadevan 2 , Yashwanth Gangadhar 2 , T N Sathyaprabha 3 , Vijay Kumavat 4 , Rose D Bharath 5 , Sanjib Sinha 1 , Arun B Taly 1
Background: The clinical spectrum of contactin-associated protein-like 2 (CASPR2) antibody-associated disease is wide and includes Morvan syndrome. Studies describing treatment and long-term outcome are limited. Aims: We report the clinical profile and emphasize response to treatment and long-term outcome in eight patients with CASPR2-antibody-associated disease. Methods: Clinical, radiological, electrophysiological, treatment, follow-up, and outcome data were collected by retrospective chart review. Results: Clinical manifestations included Morvan syndrome (n = 7) and limbic encephalitis (n = 1). None of the patients were positive for LGI1 antibody. Associated features included myasthenia (n = 1), thymoma (n = 1), and dermatological manifestations (n = 4). Patients were treated with intravenous methylprednisolone and plasma exchange during the acute symptomatic phase followed by pulsed intravenous methyl prednisolone to maintain remission. Mean-modified Rankin score at admission (pre-treatment), discharge, and last follow-up were 3.75, 2.5, and 0.42, respectively. One patient with underlying thymoma and myasthenic crisis died. The other seven patients were followed up for a mean duration of 19.71 months. All of them improved completely. Relapse occurred in one patient after 13 months but responded favorably to steroids. Conclusion: CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.
中文翻译：
CASPR2抗体相关神经系统疾病患者的临床特征和治疗反应
背景：接触素相关蛋白样 2 (CASPR2) 抗体相关疾病的临床范围很广，包括 Morvan 综合征。描述治疗和长期结果的研究是有限的。目的：我们报告了 8 名 CASPR2 抗体相关疾病患者的临床概况，并强调了对治疗和长期结果的反应。方法：通过回顾性图表审查收集临床、放射学、电生理学、治疗、随访和结果数据。结果：临床表现包括 Morvan 综合征（n = 7）和边缘脑炎（n = 1）。所有患者均未出现 LGI1 抗体阳性。相关特征包括肌无力（n = 1)、胸腺瘤 ( n = 1) 和皮肤病学表现 ( n = 4)。患者在急性症状期接受静脉注射甲基强的松龙和血浆置换治疗，然后脉冲静脉注射甲基强的松龙以维持缓解。入院（治疗前）、出院和最后一次随访时的平均修正 Rankin 评分分别为 3.75、2.5 和 0.42。一名患有潜在胸腺瘤和肌无力危象的患者死亡。其他7名患者平均随访19.71个月。全部都得到了彻底的改善。一名患者在 13 个月后复发，但对类固醇的反应良好。结论：CASPR2抗体相关疾病对免疫治疗有良好的反应，完全改善和良好的结果。潜在的恶性肿瘤可能是预后不良的标志。
更新日期：2021-04-29
Annals of Indian Academy of Neurology ( IF 1.9 ) Pub Date : 2021-03-01 , DOI: 10.4103/aian.aian_574_20 Sumanth Shivaram 1 , Madhu Nagappa 1 , Doniparthi V Seshagiri 1 , Anita Mahadevan 2 , Yashwanth Gangadhar 2 , T N Sathyaprabha 3 , Vijay Kumavat 4 , Rose D Bharath 5 , Sanjib Sinha 1 , Arun B Taly 1
Affiliation
Background: The clinical spectrum of contactin-associated protein-like 2 (CASPR2) antibody-associated disease is wide and includes Morvan syndrome. Studies describing treatment and long-term outcome are limited. Aims: We report the clinical profile and emphasize response to treatment and long-term outcome in eight patients with CASPR2-antibody-associated disease. Methods: Clinical, radiological, electrophysiological, treatment, follow-up, and outcome data were collected by retrospective chart review. Results: Clinical manifestations included Morvan syndrome (n = 7) and limbic encephalitis (n = 1). None of the patients were positive for LGI1 antibody. Associated features included myasthenia (n = 1), thymoma (n = 1), and dermatological manifestations (n = 4). Patients were treated with intravenous methylprednisolone and plasma exchange during the acute symptomatic phase followed by pulsed intravenous methyl prednisolone to maintain remission. Mean-modified Rankin score at admission (pre-treatment), discharge, and last follow-up were 3.75, 2.5, and 0.42, respectively. One patient with underlying thymoma and myasthenic crisis died. The other seven patients were followed up for a mean duration of 19.71 months. All of them improved completely. Relapse occurred in one patient after 13 months but responded favorably to steroids. Conclusion: CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.
中文翻译：
CASPR2抗体相关神经系统疾病患者的临床特征和治疗反应
背景：接触素相关蛋白样 2 (CASPR2) 抗体相关疾病的临床范围很广，包括 Morvan 综合征。描述治疗和长期结果的研究是有限的。目的：我们报告了 8 名 CASPR2 抗体相关疾病患者的临床概况，并强调了对治疗和长期结果的反应。方法：通过回顾性图表审查收集临床、放射学、电生理学、治疗、随访和结果数据。结果：临床表现包括 Morvan 综合征（n = 7）和边缘脑炎（n = 1）。所有患者均未出现 LGI1 抗体阳性。相关特征包括肌无力（n = 1)、胸腺瘤 ( n = 1) 和皮肤病学表现 ( n = 4)。患者在急性症状期接受静脉注射甲基强的松龙和血浆置换治疗，然后脉冲静脉注射甲基强的松龙以维持缓解。入院（治疗前）、出院和最后一次随访时的平均修正 Rankin 评分分别为 3.75、2.5 和 0.42。一名患有潜在胸腺瘤和肌无力危象的患者死亡。其他7名患者平均随访19.71个月。全部都得到了彻底的改善。一名患者在 13 个月后复发，但对类固醇的反应良好。结论：CASPR2抗体相关疾病对免疫治疗有良好的反应，完全改善和良好的结果。潜在的恶性肿瘤可能是预后不良的标志。
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