Anti-leucine-rich glioma-inactivated 1 encephalitis: two case reports and a review of the literature

被引:2
作者
Karvigh, Sanaz Ahmadi [1 ]
Salehizadeh, Saeideh [1 ]
Vahabizad, Fahimeh [1 ]
机构
[1] Univ Tehran Med Sci, Sina Hosp, Dept Neurol, Tehran, Iran
关键词
LGI1; Faciobrachial dystonic seizure (FBDS); Basal ganglia; Immunotherapy; Rituximab; Autoimmune encephalitis; FACIOBRACHIAL DYSTONIC SEIZURES; LGI1; IMMUNOTHERAPY; ANTIBODIES; RITUXIMAB; THERAPY; CASPR2;
D O I
10.1186/s13256-022-03650-x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background Anti-leucine-rich glioma-inactivated 1 encephalitis is a newly emerged entity characterized by frequent faciobrachial dystonic seizures and a wide spectrum of subacute clinical symptoms such as other seizure types, mood and behavioral changes, and memory loss. We should be aware of differentiating this diagnosis from psychogenic nonepileptic seizures. Mesial temporal, limbic structures, and basal ganglia are the most commonly involved regions. Case presentation Here we review the available data, and report on two young Iranian (White) females, 24 and 18 years old, who represent distinct aspects of the disease. The clinical presentation and degree of tissue involvement varies to some extent in the two reported cases. Case 1 had prominent neuropsychiatric symptoms and suffered from frequent faciobrachial dystonic seizures with more significant basal ganglia involvement, whereas case 2 suffered from severe memory decline and dialeptic seizures along with mesial temporal involvement. Symptoms were refractory to usual treatment and prompt immunotherapy was needed. Conclusions This disease has a rather favorable outcome provided that treatment is initiated early. However, resistance to first-line treatment, relapses, and long-term complications highlight the need to establish reliable biomarkers to distinguish different subtypes of this disorder to predict the clinical outcome and prognosis, and to refine management.
引用
收藏
页数:8
相关论文
共 27 条
[1]  
Bhardwaj K, 2016, J NEUROL NEUROSCI, V7, P4, DOI [10. 21767/2171-6625. 1000137, DOI 10.21767/2171-6625.1000137]
[2]   LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes [J].
Binks, Sophie N. M. ;
Klein, Christopher J. ;
Waters, Patrick ;
Pittock, Sean J. ;
Irani, Sarosh R. .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2018, 89 (05) :526-534
[3]  
Dash Deepa, 2016, BMJ Case Rep, V2016, DOI 10.1136/bcr-2016-217083
[4]   Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis [J].
de Bruijn, Marienke A. A. M. ;
van Sonderen, Agnes ;
van Coevorden-Hameete, Marleen H. ;
Bastiaansen, Anna E. M. ;
Schreurs, Marco W. J. ;
Rouhl, Rob P. W. ;
van Donselaar, Cees A. ;
Majoie, Marian H. J. M. ;
Neuteboom, Rinze F. ;
Smitt, Peter A. E. Sillevis ;
Thijs, Roland D. ;
Titulaer, Maarten J. .
NEUROLOGY, 2019, 92 (19) :E2185-E2196
[5]   Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures [J].
Flanagan, Eoin P. ;
Kotsenas, Amy L. ;
Britton, Jeffrey W. ;
McKeon, Andrew ;
Watson, Robert E. ;
Klein, Christopher J. ;
Boeve, Bradley F. ;
Lowe, Val ;
Ahlskog, J. Eric ;
Shin, Cheolsu ;
Boes, Christopher J. ;
Crum, Brian A. ;
Laughlin, Ruple S. ;
Pittock, Sean J. .
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION, 2015, 2 (06)
[6]   Expanded Phenotypes and Outcomes among 256 LGI1/CASPR2-IgG-Positive Patients [J].
Gadoth, Avi ;
Pittock, Sean J. ;
Dubey, Divyanshu ;
McKeon, Andrew ;
Britton, Jeff W. ;
Schmeling, John E. ;
Smith, Aurelia ;
Kotsenas, Amy L. ;
Watson, Robert E. ;
Lachance, Daniel H. ;
Flanagan, Eoin P. ;
Lennon, Vanda A. ;
Klein, Christopher J. .
ANNALS OF NEUROLOGY, 2017, 82 (01) :79-92
[7]  
Goldenberg Marvin M, 2012, P T, V37, P175
[8]   Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype [J].
Irani, Sarosh R. ;
Stagg, Charlotte J. ;
Schott, Jonathan M. ;
Rosenthal, Clive R. ;
Schneider, Susanne A. ;
Pettingill, Philippa ;
Pettingill, Rosemary ;
Waters, Patrick ;
Thomas, Adam ;
Voets, Natalie L. ;
Cardoso, Manuel J. ;
Cash, David M. ;
Manning, Emily N. ;
Lang, Bethan ;
Smith, Shelagh J. M. ;
Vincent, Angela ;
Johnson, Michael R. .
BRAIN, 2013, 136 :3151-3162
[9]   Faciobrachial Dystonic Seizures Precede Lgi1 Antibody Limbic Encephalitis [J].
Irani, Sarosh R. ;
Michell, Andrew W. ;
Lang, Bethan ;
Pettingill, Philippa ;
Waters, Patrick ;
Johnson, Michael R. ;
Schott, Jonathan M. ;
Armstrong, Richard J. E. ;
Zagami, Alessandro S. ;
Bleasel, Andrew ;
Somerville, Ernest R. ;
Smith, Shelagh M. J. ;
Vincent, Angela .
ANNALS OF NEUROLOGY, 2011, 69 (05) :892-900
[10]   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 [J].
Irani, Sarosh R. ;
Alexander, Sian ;
Waters, Patrick ;
Kleopa, Kleopas A. ;
Pettingill, Philippa ;
Zuliani, Luigi ;
Peles, Elior ;
Buckley, Camilla ;
Lang, Bethan ;
Vincent, Angela .
BRAIN, 2010, 133 :2734-2748