Anti-IgLON5 disease in a pediatric patient with Langerhans cell histiocytosis

Ye, Fanghua; Fan, Chenying; Peng, Min; Liu, Siqin; Yu, Yan; Yang, Lian-Yue

doi:10.1016/j.cca.2021.07.008

Cited by 8 publications

(11 citation statements)

References 16 publications

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“…However, it is clear that anti-IgLON5 disease mainly affects elder adults. Only 1 pediatric patient has ever been reported ( 11 ). Among the 162 cases, 71 were women.…”

Section: Resultsmentioning

confidence: 99%

Epileptic Seizures and Right-Sided Hippocampal Swelling as Presenting Symptoms of Anti-IgLON5 Disease: A Case Report and Systematic Review of the Literature

Zou

Liu

2022

Front. Neurol.

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Background and ObjectiveAnti-IgLON5 disease is an uncommon neurological disorder characterized by diverse clinical manifestations. Although many relevant cases have been reported, our understanding of this disorder is still quite restricted. We present a rare case of anti-IgLON5 disease and performed a comprehensive systematic review of all published cases to expand the clinical spectrum of this disorder.MethodsWe report a 61-year-old woman with an atypical presentation of epileptic seizures with abnormal signals in her right hippocampus on brain magnetic resonance imaging (MRI). A systematic review was performed of electronic databases, including PubMed, EMBASE, China National Knowledge Infrastructure (CNKI), WanFang and VIP China Science.ResultsWe identified 161 cases from 65 publications. With heterogeneous clinical manifestations, we found that bulbar dysfunction, sleep apnea, gait instability and neurocognitive and behavioral symptoms are the most common symptoms of anti-IgLON5 disease. Anti-IgLON5 antibodies presented a higher positive rate and titer in the serum than in the cerebrospinal fluid (CSF). Haplotype DRB1*10:01-DQB1*05:01 is highly correlated with anti-IgLON5 disease. Only 38 patients have presented distinctive MRI alterations (26.2%). Approximately half of the cases are responsive to immunosuppressive or immunomodulatory treatment.ConclusionAnti-IgLON5 disease is characterized by various clinical manifestations and laboratory findings. Immunotherapy may be effective in treating anti-IgLON5 disease, but the results are far from satisfactory. Studies with larger sample sizes are required to improve the current understanding of this disorder.

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“…However, it is clear that anti-IgLON5 disease mainly affects elder adults. Only 1 pediatric patient has ever been reported ( 11 ). Among the 162 cases, 71 were women.…”

Section: Resultsmentioning

confidence: 99%

Epileptic Seizures and Right-Sided Hippocampal Swelling as Presenting Symptoms of Anti-IgLON5 Disease: A Case Report and Systematic Review of the Literature

Zou

Liu

2022

Front. Neurol.

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“…The two paediatric patients in this study, one male and one female, included a case where symptoms began at 2.5 years of age, with a rapid onset and peak disease progression within approximately one week. The paediatric case reported by Ye et al (14) was diagnosed over two months post-onset.…”

Section: Discussionmentioning

confidence: 97%

Clinical analysis and literature review of two paediatric cases of anti-IgLON5 antibody-related encephalitis

Feng,

Zhou,

Kang

et al. 2024

Front. Neurol.

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IntroductionAnti-IgLON5 antibody-related encephalitis is a rare autoimmune disorder of the central nervous system, predominantly occurring in middle-aged elderly individuals, with paediatric cases being exceptionally rare. This study aims to enhance the understanding of paediatric anti-IgLON5 antibody-related encephalitis by summarising its clinical and therapeutic characteristics.MethodA retrospective analysis was conducted on two paediatric patients diagnosed with anti-IgLON5 antibody-related encephalitis at Hunan Children’s Hospital from August 2022 to November 2023. This involved reviewing their medical records and follow-up data, in addition to a literature review.ResultsThe study involved two patients, one male and one female, aged between 2.5 and 9.6 years, both presenting with an acute/subacute course of illness. Clinically, both exhibited movement disorders (including dystonia, involuntary movements, and ataxia), cognitive impairments, sleep disturbances, and psychiatric symptoms. Patient 1 experienced epileptic seizures, while Patient 2 exhibited brainstem symptoms and abnormal eye movements. Neither patient showed autonomic dysfunction. Patient 1 had normal cerebrospinal fluid (CSF) and Brain MRI findings, whereas Patient 2 showed moderate leukocytosis and mild protein elevation in the CSF, and Brain MRI revealed symmetrical lesions in the basal ganglia and cerebellum. Oligoclonal bands in the CSF were positive in both cases. Both patients tested negative for HLA-DQB*05:01 and HLA-DRB*10:01. They received both first-line and second-line immunotherapies, with Patient 2 showing a poor response to treatment.DiscussionPaediatric cases of anti-IgLON5 antibody-related encephalitis similarly present sleep disturbances as a core symptom, alongside various forms of movement disorders. Immunotherapy is partially effective. Compared to adult patients, these paediatric cases tend to exhibit more pronounced psychiatric symptoms, a more rapid onset, and more evident inflammatory changes in the CSF. The condition appears to have a limited association with HLA-DQB*05:01 and HLA-DRB*10:01 polymorphisms.

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“…Expression of the IgLON5 gene correlates with unfavorable renal cancer survival expectancy, but it is still thought that paraneoplastic activity of IgLON5 expression is rare ( 3 , 23 ). A unique case of a pediatric patient with Langerhans cell histiocytosis (LCH) and co-existing IgLON5 disease has also been described ( 24 ). A child had rapidly developed increased muscle tone during chemotherapy, presumably confirming the theory of IgLON5 having a role in maintaining an equable muscular microenvironment and its regeneration ( 24 , 25 ).…”

Section: Discussionmentioning

confidence: 99%

“…A unique case of a pediatric patient with Langerhans cell histiocytosis (LCH) and co-existing IgLON5 disease has also been described ( 24 ). A child had rapidly developed increased muscle tone during chemotherapy, presumably confirming the theory of IgLON5 having a role in maintaining an equable muscular microenvironment and its regeneration ( 24 , 25 ).…”

Section: Discussionmentioning

confidence: 99%

Anti-IgLON5 Disease – The Current State of Knowledge and Further Perspectives

et al. 2022

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Anti-IgLON5 disease is a relatively new neurological entity with the first cases reported in 2014. So far, less than 70 articles on this topic have been published. Due to its unspecific symptomatology, diverse progression, novelty and ambiguous character, it remains a difficulty for both clinical practitioners and scientists. The aim of this review is to summarize the current knowledge concerning anti-IgLON5 disease; mechanisms underlying its cause, symptomatology, clinical progression, differential diagnosis and treatment, which could be helpful in clinical practice and future research.

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Anti-IgLON5 disease in a pediatric patient with Langerhans cell histiocytosis

Cited by 8 publications

References 16 publications

Epileptic Seizures and Right-Sided Hippocampal Swelling as Presenting Symptoms of Anti-IgLON5 Disease: A Case Report and Systematic Review of the Literature

Epileptic Seizures and Right-Sided Hippocampal Swelling as Presenting Symptoms of Anti-IgLON5 Disease: A Case Report and Systematic Review of the Literature

Clinical analysis and literature review of two paediatric cases of anti-IgLON5 antibody-related encephalitis

Anti-IgLON5 Disease – The Current State of Knowledge and Further Perspectives

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