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A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate (NMDA) Receptor in Blood
Published on: January 9, 2018
[A 16-year-old boy with meningoencephalitis with auto-antibody against glutamate receptor]
Shiho Tomioka1, Masayuki Shimono, Ayako Kato
1Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Fukuoka. shihot@dp.u-netsurf.ne.jp
Abstract:
We herein report a 16-year-old boy who presented with fever and generalized convulsion. His symptoms progressively worsened, and electroencephalography (EEG) showed status with bi-frontal focus. One month after the initial presentation, examination of the cerebrospinal fluid showed pleocytosis, an increase in the IgG index and a positive oligoclonal IgG band. Brain MRI suggested slightly high signal intensity in the cerebral gray matter on FLAIR images. We considered that the patient had autoimmune meningoencephalitis, and therefore treated him with methyl-predonisolone pulse therapy, which substantially improved both his clinical condition and EEG findings. Before the therapy, the IgM and IgG auto-antibodies against glutamate receptor (GluR) epsilon 2 and delta 2 were positive, however they became negative after the therapy. In conclusion, some cases of meningoencephalitis with auto-antibodies against GluR may show an electrical status, mild brain MRI findings, and a good response to methylprednisolone pulse therapy. These clinical features were quite different from those observed for cases of Rasmussen's encephalitis.
Insights
This study details a 16-year-old with autoimmune meningoencephalitis, characterized by seizures and inflammation. Treatment with methylprednisolone pulse therapy led to significant recovery and negative auto-antibodies against glutamate receptors.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Autoimmune meningoencephalitis can present with diverse neurological symptoms.
- Glutamate receptor (GluR) antibodies are implicated in certain autoimmune encephalopathies.
- Distinguishing autoimmune meningoencephalitis from other conditions like Rasmussen's encephalitis is crucial for effective treatment.
Observation:
- A 16-year-old male presented with fever, generalized convulsions, and electroencephalography (EEG) showing status epilepticus.
- Cerebrospinal fluid analysis revealed pleocytosis, elevated IgG index, and oligoclonal bands.
- Brain MRI showed mild high signal intensity in the cerebral gray matter on FLAIR sequences.
Findings:
- The patient was diagnosed with autoimmune meningoencephalitis based on clinical, CSF, and imaging findings.
- Treatment with methylprednisolone pulse therapy resulted in substantial clinical improvement and normalization of EEG.
- Pre-treatment positivity for IgM and IgG auto-antibodies against glutamate receptor (GluR) epsilon 2 and delta 2 became negative post-therapy.
Implications:
- Autoimmune meningoencephalitis targeting GluR antibodies can manifest with electrical status and mild MRI changes.
- A positive response to methylprednisolone pulse therapy suggests an autoimmune etiology.
- These findings differentiate this condition from Rasmussen's encephalitis, aiding in diagnosis and management.
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