CASPR2-Mediated Autoimmune Encephalitis in a Toddler

Nagarajan, Balamurugan; Gowda, Vykuntaraju K; Shivappa, Sanjay K; Mahadevan, Anita

doi:10.1007/s13312-020-1928-1

Cited by 3 publications

(4 citation statements)

References 5 publications

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“…There are many cases and case series in adults but comparatively few cases in children. This review supports this standpoint, as only 38 pediatric CASPR2 antibody-associated AEs cases were retrieved [ 3 , 9 – 12 , 18 – 32 ]. It may partly explain the low prevalence of the disease in pediatric populations and limited detailed information about the condition of patients.…”

Section: Discussionsupporting

confidence: 59%

“…Twenty-one articles were included in the systematic review comprising forty (including our two cases) patients [ 3 , 9 – 12 , 18 – 32 ]. Tables 1 , 2 , 3 , and 4 in the supplement show the patient demographic, clinical characteristics, treatments, outcomes, and follow-up of CASPR2 antibody-associated AEs in children.…”

Section: Resultsmentioning

confidence: 99%

“…The few published cases of CASPR2 antibody-associated AEs in children demonstrated similar clinical features as adults, including sleep disturbances, seizures, neuropathic pain, cognitive disturbance, memory impairment, and peripheral nerve abnormalities [ 9 – 12 ]. Despite these similarities, there are significant differences between children and adults, including the most common symptoms, presence of tumors, and treatment effects.…”

Section: Introductionmentioning

confidence: 99%

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Contactin-associated protein-like 2 antibody-associated autoimmune encephalitis in children: case reports and systematic review of literature

et al. 2023

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Objectives To ascertain the clinical characteristics of pediatric patients with contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis (AEs). Methods Two cases of CASPR2 antibody-associated AEs have been reported. In addition, a systematic search of literature published between January 2010 and March 2022 through six online databases was conducted to identify the pediatric patients with CASPR2 antibody-associated AEs. Data on demographics, clinical symptoms, laboratory examinations, imaging, treatment, and outcome were collected. Results Our updated literature search yielded 1,837 publications, of which 21 were selected, and 40 patients in this study met the diagnostic criteria for AE. There were 25 males and 15 females with a mean age of 9.2 years. The most common presenting symptoms are psychiatric symptoms (72.5%), sleep changes (62.5%), and movement disorders (60%). The psychiatric symptoms included mood changes (39.1%), behavior changes (25%), and hallucination (7.5%). In total, 23 cases (57.5%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, cardiovascular-related symptoms, and sweating. No tumors were observed in children. Thirty-eight patients received first-line immunotherapy, and eight received first-line and second-line immunotherapy. All patients had a good clinical response to immune therapy. Mean mRS at onset was 3.4; It was 0.88 at the last follow-up. There was no recurrence during follow-up. Conclusion Psychiatric symptoms, sleep disorders, movement disorders, and cardiovascular-related symptoms are the most common presentation in pediatric patients with CASPR2 antibody-associated AEs. Tumor, particularly with thymoma, is uncommon in children diagnosed with CASPR2 antibody-associated AEs. In addition, prompt diagnosis and immunotherapy can relieve symptoms and improve the prognosis. Supplementary Information The online version contains supplementary material available at 10.1007/s13760-023-02174-5.

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Section: Discussionsupporting

confidence: 59%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Contactin-associated protein-like 2 antibody-associated autoimmune encephalitis in children: case reports and systematic review of literature

et al. 2023

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“…4 A case of CASPR2-mediated autoimmune limbic encephalitis in a 19-month-old toddler in India has been recently described. 5 In 1 of the recently reported case series, encephalopathy, seizures, and PNH were the commonly reported symptoms. 6 Movement disorders associated with CASPR2 antibodies are rare and underreported.…”

mentioning

confidence: 98%

Contactin‐Associated Protein‐Like 2 (CASPR2)–Associated Movement Disorder in a Child

Nagireddy

Kumar

Joshi

2021

Movement Disord Clin Pract

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Anti-contactin-associated protein-like 2 antibody-associated encephalitis in children: A case report and literature review

Dou

Shu

2022

Front. Pediatr.

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BackgroundAnti-Contactin-associated protein-like 2 (CASPR2) antibody-associated encephalitis is a rare group of autoimmune diseases that causes extensive damage to the central and/or peripheral nervous system.Case presentationHere, we reported a case of anti-CASPR2 antibody-associated encephalitis in a 12-year-old male patient with symptoms of headache, consciousness disturbance, mental abnormalities, urinary incontinence, fasciculations in the extremity muscles, and involuntary movements. The testing for autoimmune encephalitis-associated antibodies showed that CASPR2-associated antibodies were positive, and electroencephalography showed diffuse slow waves. No tumor was found after screening for malignancies. The child's status significantly improved after receiving immunotherapy with intravenous methylprednisolone and immunoglobulin.ConclusionsAnti-CASPR2 antibody-associated encephalitis has been rarely reported in children. It has a complex clinical presentation and a low incidence of tumor. Most pediatric patients have a favorable prognosis and relapse is uncommon.

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CASPR2-Mediated Autoimmune Encephalitis in a Toddler

Cited by 3 publications

References 5 publications

Contactin-associated protein-like 2 antibody-associated autoimmune encephalitis in children: case reports and systematic review of literature

Contactin-associated protein-like 2 antibody-associated autoimmune encephalitis in children: case reports and systematic review of literature

Contactin‐Associated Protein‐Like 2 (CASPR2)–Associated Movement Disorder in a Child

Anti-contactin-associated protein-like 2 antibody-associated encephalitis in children: A case report and literature review

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