Anti-N-methyl-D-aspartate receptor encephalitis: a newly recognized inflammatory brain disease in children
Bibliographic record
Abstract
Over an 18-month period (July 2009-Dec 2010), consecutive children presenting with newly acquired psychiatric and/or neurologic deficits consistent with anti-NMDAR encephalitis and evidence of central nervous system (CNS) inflammation were screened. Serum and cerebrospinal fluid (CSF) samples were obtained and sent for anti-NMDAR antibody testing. Children were included in the study if they had confirmatory evidence of these antibodies in the serum and/or CSF. Details of clinical presentation and results of investigations, including inflammatory markers, CSF studies and neuroimaging, were documented. Type and duration of treatment and outcomes at last follow-up were evaluated. Over the study period a total of 18 children presented with clinical features compatible with anti-NMDAR encephalitis including psychiatric manifestations, seizures and/or movement disorders. Four children (22%) had positive anti-NMDAR antibodies and were diagnosed with anti-NMDAR encephalitis. These included one male and three females, with a median age of 12.8 years (range 3-16 years). The remaining 14 children were subsequently diagnosed with 1) primary CNS vasculitis (4); 2) post-infectious inflammatory brain disease (2); 3) channelopathy (1); 4) reversible splenial lesions syndrome (1); 5) epilepsy (1); and 6) inflammatory brain disease NYD (5). All children with anti-NMDAR encephalitis presented with neuropsychiatric deficits including, seizures, speech disorder, sleep disturbance, and fluctuating level of consciousness. The three older patients also had prominent psychiatric features, while the younger child had significant autonomic instability and prominent involuntary movement disorder. None had an underlying tumor. Immunosuppressive therapy with intravenous immunoglobulin, steroids and/or rituximab, resulted in near or complete recovery; however, two of the patients had early relapse requiring re-treatment. Anti-NMDAR encephalitis is an important, reversible cause of neuropsychiatric deficits in children that must be included in the differential diagnosis of CNS vasculitis and other inflammatory brain diseases. Early diagnosis and treatment are essential for neurologic recovery.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".