GABA-A receptor antibody encephalitis

Etiology

• paraneoplastic neurologic syndrome*
  • marginal zone lymphoma

* may or may not be associated malignancy or malignancy may be in remission

Pathology

• inflammatory changes consistent with reactive gliosis

Clinical manifestations

• seizures
  • status epilepticus
  • seizures may recur after resolution of status epilepticus
• no focal neurological deficits

Laboratory

• CSF analysis
  • no pleocytosis
  • normal CSF protein
  • negative CSF oligoclonal banding

Diagnostic procedures

• 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

Management

• intravenous methylprednisolone & plasmapheresis for 5 days
• maintenance with rituximab

More general terms

• paraneoplastic neurologic syndrome
• encephalitis

Additional terms

• GABA-A receptor