Morvan Syndrome Converted from Isaacs? Syndrome after Thymectomy with Positivity for Both Anti-LGI1 and Anti-CASPR2 Antibodies

被引:1
作者
Suzuki, Daisuke [1 ]
Suzuki, Yoshihiro [1 ]
Sato, Daisuke [1 ]
Kikuchi, Kenji [1 ]
Kanauchi, Naoki [2 ]
Nishida, Akiko [3 ]
Ohta, Yasuyuki [4 ]
机构
[1] Nihonkai Gen Hosp, Dept Neurol, Sakata, Yamagata, Japan
[2] Nihonkai Gen Hosp, Dept Resp Surg, Sakata, Yamagata, Japan
[3] Nihonkai Gen Hosp, Dept Pathol, Sakata, Yamagata, Japan
[4] Yamagata Univ, Dept Internal Med 3, Div Neurol & Clin Neurosci, Fac Med, Yamagata, Japan
关键词
CASPR2; Isaacs? syndrome; LGI1; Morvan syndrome; thymectomy;
D O I
10.2169/internalmedicine.8145-21
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Anti-voltage-gated potassium channel complex antibodies-mediated disorder includes Isaacs??? syndrome, which is characterized by neuromyotonia, and Morvan syndrome, which is characterized by neuromyotonia, encephalopathy and autonomic dysfunction. We herein report a patient with Morvan syndrome that converted from Isaacs??? syndrome after thymectomy. The patient first presented with myospasm in all extremities and positivity for both anti-leucine-rich glioma inactivated 1 (LGI1) and anti-contactin-associated protein like 2 (CASPR2) antibodies and subsequently developed encephalopathy after thymectomy, which was successfully improved by immunotherapy. This is the first case of Morvan syndrome wherein thymectomy worsened Isaacs??? syndrome, suggesting that immunotherapy should be considered for Isaacs??? syndrome accompanied by positivity for both anti-LGI1 and anti-CASPR2 antibodies to prevent worsening to Morvan syndrome.
引用
收藏
页码:1443 / 1445
页数:3
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