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Paroxysmal EEG pattern in a child with N-methyl-D-aspartate receptor antibody encephalitis
Svetlana Gataullina1, Perrine Plouin, Angela Vincent
1Neuropaediatrics Department, Hôpital Necker-Enfants Malades, Paris, France.
Abstract:
A previously healthy 8-year-old male presented with cognitive regression, sleep disturbance, hallucinations, and severe attacks of agitation and oligoclonal bands in the cerebrospinal fluid. N-methyl-d-aspartate receptor (NMDAR) antibodies in serum and cerebrospinal fluid were detected 2 months after onset of symptoms. Bursts of agitation were initially considered to be epileptic leading to the administration of a high dose of benzodiazepines. Video-electroencephalography (EEG) failed to disclose any correlation between the episodes of agitation and paroxysmal rhythmic slow activity on EEG persisting throughout and after attacks of agitation. Clinical improvement and EEG normalization followed an initial plasma exchange performed 3 months after onset of disease. This particular paroxysmal EEG pattern in NMDAR antibody encephalitis suggests that it may result from the combination of reduced NMDAR function and major γ-aminobutyric acid (GABA)-ergic activation.
Insights
A rare autoimmune encephalitis linked to N-methyl-d-aspartate receptor (NMDAR) antibodies caused severe neurological symptoms in a child. Plasma exchange led to clinical and EEG improvement, suggesting a novel EEG pattern in NMDAR antibody encephalitis.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Autoimmune encephalitis is a severe neurological disorder.
- N-methyl-d-aspartate receptor (NMDAR) antibodies are implicated in a subset of these cases.
- Early diagnosis and treatment are crucial for patient outcomes.
Observation:
- An 8-year-old boy presented with cognitive regression, hallucinations, and agitation.
- Cerebrospinal fluid analysis revealed oligoclonal bands, and NMDAR antibodies were detected in serum and CSF.
- Epilepsy was suspected due to agitation, but EEG showed no correlation.
Findings:
- A unique paroxysmal EEG pattern was observed during agitation episodes.
- This pattern persisted despite the absence of epileptic activity.
- Treatment with plasma exchange resulted in clinical recovery and EEG normalization.
Implications:
- The observed EEG pattern may indicate a specific pathophysiological mechanism in NMDAR antibody encephalitis.
- This pattern might reflect a combination of reduced NMDAR function and GABAergic activation.
- Understanding this EEG pattern could aid in earlier diagnosis and targeted treatment strategies.
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