Potassium channel-complex antibodies associated limbic encephalitis_Journal of Epilepsy

Authors：

 HUXiang-shu ¹ , OUYang-mei ¹ , LIHua ¹ , WUXin-yan ¹ , CHENQiao ¹ , YANGLi ¹ , XUEShao-qiang ²

1. Epileptic Center, Guangdong 999 Brain Hospital, Guangzhou 510510, Guangdong, China;
2. Laboratory Department of Guangdong 999 Brain Hospital, Guangzhou 510510, Guangdong, China;

Corresponding author：

HUXiang-shu, Email: hxs75@126.com

Keywords：

Potassium channel-complex antibodies; Limbic encephalitis; Clinical features

DOI：

10.7507/2096-0247.20150003

Video：

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Objective To make a better understanding of potassium channel-complex autoimmune antibodies associated limbic encephalitis, we studied in details with patients of this autoimmune disease accompanying without tumors. Methods Diagnosis of 3 patients were confirmed by antibody detection in serum or CSF. All the clinical data, including history, CSF data, cranial MRI, EEG, pelvic ultrasound and treatment strategy, were carefully gathered. Two to eleven months follow-up were carried out. Results 3 female adult patients showed common initial manifestation of seizures, and changes of consciousness, mental disorder and cognitive impairment. Hyponatremia was found in one LGI1-Ab+ patient. Cranial MRI showed unilateral or bilateral signal changes with limbic system. Changes of CSF and EEG were nonspecific. All 3 patients became recovery in different levels after two to eleven months. Conclusions Potassium channel-complex antibodies associated encephalitis may be a common type of limbic encephalitis in adults without tumors. Seizures may be the first sign of the disease. Hyponatremia is one of characteristics of LGI1-Ab+ patient. Patients may have a fairly good short outcome.

Citation： HUXiang-shu, OUYang-mei, LIHua, WUXin-yan, CHENQiao, YANGLi, XUEShao-qiang. Potassium channel-complex antibodies associated limbic encephalitis. Journal of Epilepsy, 2015, 1(1): 22-27. doi: 10.7507/2096-0247.20150003 Copy

1.	籍炀飞, 张博爱, 贾延劼. 电压门控钾通道自身抗体相关性边缘性脑炎. 中华神经科杂志, 2008, 8(41):560-562.
2.	Asztely F, Kumlien E. The diagnosis and treatment of limhic encephalitis. Aeta Neurol Scand, 2012, 126(33):365-375.
3.	Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. Brain, 2010, 133(16):2734-2748.
4.	Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1-antibody limbic encephalitis. Ann Neurol, 2011, 69(24):892-900.
5.	Malter MP, Frisch C, Schoene-Bake JC, et al. Outcome of limbic encephalitis with VGKC-complex antibodies:relation to antigenic specificity. J Neurol, 2014,261(131):1695-1705.
6.	Irania SR, Bien CG. Autoimmune epilepsies. Curr Opin Neurol, 2011, 24(6):146-153.
7.	Vincent A. Developments in autoimmune channelopathies. Autoimmu Rev, 2013, 12(6):678-681.
8.	郑艺明, 孙葳, 王朝霞, 等. 富亮氨酸胶质瘤失活1蛋白A自身抗体相关边缘性脑炎1例. 北京大学学报(医学版), 2014, 46(4):646-649.
9.	Lai M, Huijbers GM M, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels:a case series. Lancet Neurol, 2010, 9(8):776-785.
10.	Lancaster E, Huijbers M, Bar V, et al. Investigations of Caspr2, an autoantigen of encephalitis and neuromyotonia. Ann Neurol, 2011, 69(2):303-311.
11.	Jarius S, Hoffmann L, Clover L, et al. CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis. J Neurol Sci, 2008, 268(1-2):74-77.
12.	Vincent A, Buckley C, Schott JM, et al. Potassium channel antibody-associated encephalopathy:a potentially immunotherapy-responsive form of limbic encephalitis. Brain, 2004, 127(3):701-712.
13.	Hiraga A, Kuwabara S, Hayakawa S, et al. Voltage-gated potassium channel antibody associated encephalitis with basal ganglia lesions. Neurology, 2006, 66(23):1780-1781.
14.	Hacohen Y, Wright S, Siddiqui A, et al. A clinico-radiological phenotype of voltage-gated potassium channel complex antibody-mediated disorder presenting with seizures and basal ganglia changes. Develop Med Child Neurol, 2012, 54(22):1157-1159.

1. 籍炀飞, 张博爱, 贾延劼. 电压门控钾通道自身抗体相关性边缘性脑炎. 中华神经科杂志, 2008, 8(41):560-562.
2. Asztely F, Kumlien E. The diagnosis and treatment of limhic encephalitis. Aeta Neurol Scand, 2012, 126(33):365-375.
3. Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. Brain, 2010, 133(16):2734-2748.
4. Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1-antibody limbic encephalitis. Ann Neurol, 2011, 69(24):892-900.
5. Malter MP, Frisch C, Schoene-Bake JC, et al. Outcome of limbic encephalitis with VGKC-complex antibodies:relation to antigenic specificity. J Neurol, 2014,261(131):1695-1705.
6. Irania SR, Bien CG. Autoimmune epilepsies. Curr Opin Neurol, 2011, 24(6):146-153.
7. Vincent A. Developments in autoimmune channelopathies. Autoimmu Rev, 2013, 12(6):678-681.
8. 郑艺明, 孙葳, 王朝霞, 等. 富亮氨酸胶质瘤失活1蛋白A自身抗体相关边缘性脑炎1例. 北京大学学报(医学版), 2014, 46(4):646-649.
9. Lai M, Huijbers GM M, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels:a case series. Lancet Neurol, 2010, 9(8):776-785.
10. Lancaster E, Huijbers M, Bar V, et al. Investigations of Caspr2, an autoantigen of encephalitis and neuromyotonia. Ann Neurol, 2011, 69(2):303-311.
11. Jarius S, Hoffmann L, Clover L, et al. CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis. J Neurol Sci, 2008, 268(1-2):74-77.
12. Vincent A, Buckley C, Schott JM, et al. Potassium channel antibody-associated encephalopathy:a potentially immunotherapy-responsive form of limbic encephalitis. Brain, 2004, 127(3):701-712.
13. Hiraga A, Kuwabara S, Hayakawa S, et al. Voltage-gated potassium channel antibody associated encephalitis with basal ganglia lesions. Neurology, 2006, 66(23):1780-1781.
14. Hacohen Y, Wright S, Siddiqui A, et al. A clinico-radiological phenotype of voltage-gated potassium channel complex antibody-mediated disorder presenting with seizures and basal ganglia changes. Develop Med Child Neurol, 2012, 54(22):1157-1159.

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