Anti-LGI1 encephalitis

Sonderen, Agnes van; Thijs, Roland D.; Coenders, Elias C.; Jiskoot, Lize C.; Sánchez, Esther; Bruijn, Marienke A.A.M. de; Coevorden-Hameete, Marleen H. van; Wirtz, Paul W.; Schreurs, Marco W. J.; Smitt, Peter A.E. Sillevis; Titulaer, Maarten J.

doi:10.1212/wnl.0000000000003173

Cited by 498 publications

(421 citation statements)

References 31 publications

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“…Our patient tested positive for LGI1 in the serum only, illustrating that, in this entity, some patients may have antibodies only detectable in the serum, 5 whereas others may have antibodies only detectable in the CSF. 6 …”

Section: Discussionmentioning

confidence: 70%

Striking basal ganglia imaging abnormalities in LGI1 ab faciobrachial dystonic seizures

Chiriboga

Siegel

Tatum

et al. 2017

Neurol Neuroimmunol Neuroinflamm

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Section: Discussionmentioning

confidence: 70%

Striking basal ganglia imaging abnormalities in LGI1 ab faciobrachial dystonic seizures

Chiriboga

Siegel

Tatum

et al. 2017

Neurol Neuroimmunol Neuroinflamm

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“…Two-thirds of patients present with brain MRI hyperintensities in the medial temporal lobe (Figure 2). Paraneoplastic anti-LGI1 encephalitis is uncommon; however, patients should be screened for lung cancer 27 . Relapses may occur in up to 20% of patients 28 .…”

Section: Anti-lgi1 and Anti-caspr2 Encephalitis (Formerly Known As Anmentioning

confidence: 99%

“…Nearly one-third of patients develop Morvan's syndrome, a complex disorder affecting the peripheral and central nervous system that is characterized by distal movement disorders of the upper limbs, peripheral nerve hyperexcitability, dysautonomia, pain, and encephalitis 24 . Most individuals affected are male and one-third of them present with paraneoplastic manifestations, usually associated with thymoma, lung cancer or endometrial carcinoma 27,26 .…”

Section: Anti-lgi1 and Anti-caspr2 Encephalitis (Formerly Known As Anmentioning

confidence: 99%

Autoimmune encephalitis: a review of diagnosis and treatment

Dutra

Abrantes

Toso

et al. 2018

Arq. Neuro-Psiquiatr.

107

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Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.

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“…These antibodies are in fact directed against two neuronal cell-surface proteins, leucine-rich glioma inactivated-1 (LGI1) and contactin-associated protein-2 receptor (CASPR2), [36,37] and several unknown (probably intracellular) proteins. Whereas LGI1 or CASPR2 antibodies associate with several syndromes that often occur with seizures (e.g., limbic encephalitis, Morvan syndrome), [38**,39*,40**,41*] the detection of VGKC-complex antibodies negative for LGI1 and CASPR2 has unclear clinical utility. [42,43**]…”

Section: Introductionmentioning

confidence: 99%

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Spatola

Dalmau

2017

Current Opinion in Neurology

152

137

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Purpose of review To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Recent findings Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Summary Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.

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Anti-LGI1 encephalitis

Cited by 498 publications

References 31 publications

Striking basal ganglia imaging abnormalities in LGI1 ab faciobrachial dystonic seizures

Striking basal ganglia imaging abnormalities in LGI1 ab faciobrachial dystonic seizures

Autoimmune encephalitis: a review of diagnosis and treatment

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

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