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Published on: October 28, 2022
Pediatric anti-NMDAR encephalitis: Neuroradiological presentation, cognitive outcome and volumetric MRI analysis
Fabio M Doniselli1, Anna Paola Savoldi1, João Ramos2
1Neuroradiology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Abstract:
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most frequent autoimmune encephalitis in children and may cause long-term sequelae. Although MRI plays a key role in diagnosis and follow-up, its correlation with cognitive outcome remains unclear. We retrospectively reviewed pediatric cases of anti-NMDAR encephalitis diagnosed from 2007 to 2024, including only antibody-positive patients with at least one MRI. Clinical data encompassed presenting symptoms, EEG, therapies, relapses, and the latest neuropsychological assessment. MRI scans were evaluated by three neuroradiologists using a structured atrophy scale, and volumetric T1-weighted sequences, when available, were analyzed with Volbrain. Twenty-two patients (9 males, 13 females; median onset age 11.9 years) were included. The most frequent presenting symptoms were seizures (82%) and behavioral disturbances (59%), while EEG abnormalities were detected in 95%. At onset, 9 patients (41%) showed MRI abnormalities, most commonly fronto-parieto-occipital atrophy. Long-term cognitive follow-up (median 4.8 years), available for all patients, demonstrated that 80% of patients had normal or only mild deficits, but those with abnormal baseline MRI were more likely to develop long-term cognitive impairment, although the limited sample size precluded robust statistical analysis. Long-term MRI was available in 8 patients (median follow-up 10 years); volumetric analysis revealed parietal lobe volume loss in 6, including some with visually normal scans. Overall, baseline MRI alterations appeared associated with higher risk of subsequent cognitive deficits, while volumetric data highlighted subtle parietal atrophy even in patients with normal-appearing scans and, in some cases, without neuropsychological impairment. These findings support the complementary role of quantitative MRI and systematic neuropsychological monitoring in pediatric anti-NMDAR encephalitis.
Insights
Pediatric anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis can cause cognitive issues. Abnormal baseline MRI in children with anti-NMDAR encephalitis may predict long-term cognitive impairment, even with subtle atrophy.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a leading cause of autoimmune encephalitis in children, potentially leading to lasting neurological deficits.
- Magnetic Resonance Imaging (MRI) is crucial for diagnosing and monitoring anti-NMDAR encephalitis, but its direct correlation with long-term cognitive outcomes in pediatric patients requires further investigation.
Purpose of the Study:
- To investigate the relationship between baseline and long-term MRI findings and cognitive outcomes in pediatric patients diagnosed with anti-NMDAR encephalitis.
- To assess the utility of quantitative MRI analysis in identifying subtle brain changes associated with cognitive sequelae.
Main Methods:
- Retrospective review of 22 pediatric anti-NMDAR encephalitis cases (2007-2024) with antibody confirmation and at least one MRI scan.
- Evaluation of MRI scans by three neuroradiologists using a structured atrophy scale and volumetric analysis (Volbrain) of T1-weighted sequences.
- Analysis of clinical data including presenting symptoms, EEG, therapies, relapses, and long-term neuropsychological assessments (median follow-up 4.8 years).
Main Results:
- Most pediatric anti-NMDAR encephalitis patients (80%) achieved normal or mild cognitive deficits long-term.
- Patients with abnormal baseline MRI findings were more likely to experience long-term cognitive impairment.
- Volumetric MRI analysis revealed parietal lobe volume loss in 6 of 8 patients with long-term scans, including some with visually normal scans and no apparent cognitive deficits.
Conclusions:
- Baseline MRI abnormalities in pediatric anti-NMDAR encephalitis are associated with an increased risk of subsequent cognitive deficits.
- Quantitative MRI, particularly volumetric analysis, can detect subtle parietal atrophy even in patients with normal-appearing scans, highlighting its complementary role in monitoring.
- Systematic neuropsychological monitoring alongside quantitative MRI is recommended for comprehensive management of pediatric anti-NMDAR encephalitis.
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