An immunologic case study of acute encephalitis with refractory, repetitive partial seizures

Area of Science:

• Neurology
• Immunology
• Neuroimmunology

Background:

• Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a severe neurologic syndrome.
• AERRPS is characterized by frequent, refractory partial seizures that progress to epilepsy.

Purpose of the Study:

• To investigate the pathophysiology of AERRPS.
• To explore the immunologic underpinnings of AERRPS.

Main Methods:

• Immunologic assessment of a patient with AERRPS.
• Analysis of peripheral blood mononuclear cells for natural killer (NK) cell activity.
• Detection of IgG antibodies against glutamate receptor (GluR) subunits in serum and cerebrospinal fluid (CSF).
• Measurement of plasma and CSF cytokine concentrations (IL-2, IL-6, IL-10, TNF-α, IFN-γ).

Main Results:

• Decreased NK cell activity was observed in peripheral blood mononuclear cells.
• Positive IgG antibodies against GluRε2, ζ1, and δ2 subunits were found in serum and CSF.
• Elevated plasma concentrations of IL-2, IL-6, IL-10, TNF-α, and IFN-γ were detected.
• Extremely high CSF levels of IL-6 were measured.

Conclusions:

• AERRPS is likely an immune-mediated encephalitis.
• Both autoimmunity and exaggerated cytokine production are implicated in AERRPS pathogenesis.
• NK cell dysfunction may be a key factor contributing to GluR autoantibody production in AERRPS.