Pediatric LGI1 and CASPR2 autoimmunity associated with COVID 19: Morvan syndrome

Kılıç, Mehmet Akif; Yildirim, Zeynep Nagihan Yoruk; Oner, Adil; Yesil, Elif; Aksu, Bağdagül; Yıldız, Edibe Pembegül; Yılmaz, Alev; Caliskan, Meliha Mine

doi:10.1007/s00415-021-10614-6

Cited by 8 publications

(10 citation statements)

References 13 publications

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“…These 61 articles comprised 47 on COVID-19-associated AIE, 1 case report on possible Bickerstaff encephalitis, 1 case series on both of the above subgroups, and 12 on vaccine-associated AIE. The articles included 52 case reports 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 and 9 case series. 5 6 67 68 69 70 71 72 73 Fig.…”

Section: Resultsmentioning

confidence: 99%

“…Rare reports of antineuronal-antibody-associated AIE included two cases of CASPR2 30 71 and single reports of antimyelin, 69 LGI-1, 39 GFAP, 34 amphiphysin, 58 Yo, 69 and a novel antibody detected against mouse brain neuronal proteins 24 associated with AIE. All of the cases showed a temporal association with SARS-CoV-2 infection.…”

Section: Resultsmentioning

confidence: 99%

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AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

et al. 2022

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Background and Purpose Autoimmune encephalitis (AIE) following coronavirus disease 2019 (COVID-19) is an underexplored condition. This study aims to systematically review the clinico-investigational and pathophysiologic aspects of COVID-19 and its vaccines in association with AIE, and identify the factors predicting neurological severity and outcomes. Methods Relevant data sources were searched using appropriate search terms on January 15, 2022. Studies meeting the criteria for AIE having a temporal association with COVID-19 or its vaccines were included. Results Out of 1,894 citations, we included 61 articles comprising 88 cases: 71 of COVID-19-associated AIE, 3 of possible Bickerstaff encephalitis, and 14 of vaccine-associated AIE.There were 23 definite and 48 possible seronegative AIE cases. Anti-NMDAR (N-methyl-D-aspartate receptor; n =12, 16.9%) was the most common definite AIE. Males were more commonly affected (sex ratio=1.63) in the AIE subgroup. The neurological symptoms included alteredmental state ( n =53, 74.6%), movement disorders ( n =28, 39.4%), seizures ( n =24, 33.8%), behavioural ( n =25, 35.2%), and speech disturbances ( n =17, 23.9%). The median latency to AIE diagnosis was 14 days (interquartile range=4–22 days). Female sex and ICU admission had higherrisks of sequelae, with odds ratio (OR) of 2.925 (95% confidence interval [CI]=1.005–8.516)and 3.515 (95% CI=1.160–10.650), respectively. Good immunotherapy response was seen in42/48 (87.5%) and 13/13 (100%) of COVID-19-associated and vaccine-associated AIE patients, respectively. Sequelae were reported in 22/60 (36.7%) COVID-19 associated and 10/13 (76.9%) vaccine-associated cases. Conclusions The study has revealed diagnostic, therapeutic, and pathophysiological aspects of AIE associated with COVID-19 and its vaccines, and its differences from postinfectious AIE. Systematic review registration PROSPERO registration number CRD42021299215

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

et al. 2022

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“…Fifteen patients were collected from seven publications ( 5 , 6 , 9 , 12 – 15 ). Together with the 2 patients we presented, a total of 17 pediatric patients were identified, including 12 males and 5 females.…”

Section: Resultsmentioning

confidence: 99%

“…To date, no more than 70 cases of double-positive antibodies have been reported in adults and children ( 2 , 6 , 15 , 16 ). In the study, we describe two pediatric Morvan syndrome with LGI1-CASPR2 double-positive and present the results of the first systematic literature review on pediatric LGI1-CASPR2 double-positive cases.…”

Section: Discussionmentioning

confidence: 99%

Phenotypic Spectrum of CASPR2 and LGI1 Antibodies Associated Neurological Disorders in Children

Jiang

Tan

et al. 2022

Front. Pediatr.

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ObjectivesThe clinical data of patients with double-positive for leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies is limited, particularly for children. This study aimed to investigate and summarize the clinical features and long-term prognosis of children’s LGI1 and CASPR2 antibodies related to neurological disorders.MethodsWe collected the clinical data and prognosis of patients with dual positive antibodies of CASPR2 and LGI1, hospitalized in the Department of Neurology, Children’s Hospital of Chongqing Medical University. Furthermore, we summarized the clinical phenotypes of this disorder in children by reviewing the published literature.ResultsTwo patients presenting with variable neurological symptoms including pain, hypertension, profuse sweating, irritability, and dyssomnia from Children’s Hospital of Chongqing Medical University were enrolled in this study. Together with the two patients, we identified 17 children with dual CASPR2 and LGI1 antibodies, including 12 males and 5 females. At the onset, the median age was 4.1 years (range 1–16, interquartile range 2.5–13.5), with 9 children younger than 5 years and 6 adolescents. Of the 17 patients, 11 were diagnosed with Morvan syndrome, 4 with acquired neuromyotonia, 1 with Guillain-Barré syndrome, and 1 with Guillain-Barré syndrome combined with Morvan syndrome. Dysautonomia (14/17, 82.3%), pain (13/17, 76.4%), sleep disorders (13/17, 76.4%), encephalopathy (12/17, 70.5%), and weight loss (10/17, 58.8%) were the most frequently described symptoms overall. No tumors were identified. Of the 17 patients, 13 received immunotherapy comprising IVIG combination of IVMP during the acute symptomatic phase followed by oral prednisolone to maintain remission (n = 7), the combination of IVIG, IVMP, oral prednisolone and methotrexate (n = 1), the combination of IVIG, IVMP, and mycophenolate mofetil (n = 1), the combination of IVIG, IVMP, oral prednisolone, and rituximab (n = 1), IVIG only (n = 2), IVMP only (n = 1). Median modified Rankin Scale (mRS) scores in the acute phase were 3 (range 1–4) and improved gradually. Over the follow-up (median 8.6 months, range 1–36 months), 52.9% (9/17) of the patients recovered completely; one patient relapsed and showed immunotherapy-dependent.ConclusionLGI1 and CASPR2 double-positive antibodies associated with the neurological diseases can occur in children of all ages and involve multiple nervous systems. Morvan syndrome is the most common phenotype of this disorder. The long-term outcomes are mostly favorable upon immunotherapy.

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“…Autoantibodies directed against Purkinje cells, striatal neurons, and hippocampal neurons have been reported 85 . Other antibodies reported in the setting of COVID‐19 infection include LGI‐1, 47 NMDAR, 86 CASPR‐2, 87 MOG, 88 and GAD65 44 . We must consider the possibility that these antibodies are an epiphenomenon rather than directly causative, particularly when the clinical picture does not fit with the well‐elucidated spectrum of those autoantibodies.…”

Section: The Emergence Of Movement Disorders Following Sars‐cov‐2 Inf...mentioning

confidence: 99%

Movement Disorders andSARS‐CoV‐2

Fung

Fasano

Fearon

2022

Movement Disord Clin Pract

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Pediatric LGI1 and CASPR2 autoimmunity associated with COVID 19: Morvan syndrome

Cited by 8 publications

References 13 publications

AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

AI-CoV Study: Autoimmune Encephalitis Associated With COVID-19 and Its Vaccines—A Systematic Review

Phenotypic Spectrum of CASPR2 and LGI1 Antibodies Associated Neurological Disorders in Children

Movement Disorders andSARS‐CoV‐2

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