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GABA-A receptor antibody encephalitis
Etiology:
- paraneoplastic neurologic syndrome*
- marginal zone lymphoma [1]
* may or may not be associated malignancy or malignancy may be in remission [1]
Pathology:
- inflammatory changes consistent with reactive gliosis
Clinical manifestations:
- seizures
- status epilepticus [1]
- seizures may recur after resolution of status epilepticus
- no focal neurological deficits
Laboratory:
- CSF analysis
- no pleocytosis
- normal CSF protein
- negative CSF oligoclonal banding
Special laboratory:
- brain biopsy
Radiology:
- MRI of brain without gadolinium contrast
- hyperintense lesions on T2 FLAIR sequence in right & left inferior frontal lobes
- new hyperintense lesions on T2 FLAIR sequence may appear after resolution of earlier lesions
* images [1]
Management:
- intravenous methylprednisolone & plasmapheresis for 5 days [1]
- maintenance with rituximab [1]
Related
GABA-A receptor
General
paraneoplastic neurologic syndrome
encephalitis
References
- Baqal O, Vanood A, Harahsheh E
Magnetic Resonance Imaging Features of GABA-A Receptor Antibody-Mediated
Encephalitis.
JAMA Neurol. Published online February 6, 2023.
PMID: 36745432
https://jamanetwork.com/journals/jamaneurology/fullarticle/2801291