Highlights
- •A paediatric case of GABAAR encephalitis without development of seizures despite severe phenotype.
- •A full recovery upon early immunosuppression unlike previous reports.
- •A report extending the phenotype of this rare disease.
- •A medical history raising questions about the aetiology of autoimmune encephalitis.
Abstract
Background
Autoimmune-mediated processes are the driving force behind many neurological diseases.
Autoimmune encephalitis, a group of syndromes, mediated by or at least associated
with autoantibodies against neuronal tissue, have gained increasing importance especially
in paediatric neurology. Since the first NMDAR encephalitis was described a growing
number of patients with encephalopathy, seizures and psychiatric symptoms were found
to suffer from treatable autoimmune disorders. Recently a severe form of encephalitis
associated with GABAAR antibodies was described showing extensive MRI abnormalities and refractory seizures.
Case
We now describe a child with catatonia and encephalopathy due to antibodies against
the GABAA receptor. It is a rare paediatric case without the development of seizures despite
severe encephalopathy.
Relevance
The report extends the phenotype of this rare disease. It demonstrates a favourable
outcome after introduction of an early and aggressive immunomodulatory therapy. Due
to the child's history of previous viral meningitis, the case raises questions about
the unrevealed mechanisms leading to autoimmune encephalitis, including the model
of a viral trigger as discussed in Herpes infection and NMDAR encephalitis. Finally,
it describes in detail the neuropsychological symptoms and cognitive functions during
disease flare and recovery.
Keywords
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Article info
Publication history
Published online: January 09, 2018
Accepted:
January 2,
2018
Received in revised form:
November 22,
2017
Received:
May 12,
2017
Identification
Copyright
© 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
