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Efficacy of Tocilizumab in Limbic Encephalitis with Anti-CASPR2 Antibodies
Maurizio Benucci1, Luciana Tramacere2, Maria Infantino3
1Rheumatology Unit, S. Giovanni di Dio Hospital, Florence, Italy.
Abstract:
We report the case of a 64-year-old man who presented with subacute memory, balance impairment, behavioral and mood changes, and epileptic seizures. Magnetic resonance imaging (MRI) showed bilateral hippocampal abnormalities. Brain [18F]-FDG fluorodeoxyglucose positron emission tomography (PET) revealed hypometabolism in both the temporal lobe as well as in the left insular and parietal regions. The clinical and neuroradiological picture and the detection of anti-CASPR2 antibodies in serum oriented the diagnosis towards autoimmune limbic encephalitis. Intravenous high-dose steroid and immunoglobulin treatments were ineffective. We did not use rituximab for the presence of antibodies to HbcAg positivity. Tocilizumab given intravenously 8 mg/kg once a month for six months and then subcutaneously 162 mg every week for six months resulted in clinical and neuroradiological improvement. These data support the efficacy of tocilizumab in autoimmune limbic encephalitis associated with anti-CASPR2 antibodies, which has been sporadically reported in the literature.
Insights
Tocilizumab effectively treated autoimmune limbic encephalitis (ALE) in a patient with anti-CASPR2 antibodies. This offers a new therapeutic option for this rare neurological disorder.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Autoimmune limbic encephalitis (ALE) is a rare neurological disorder characterized by inflammation of the limbic system.
- CASPR2 antibodies are implicated in a subset of ALE cases, often presenting with cognitive and psychiatric symptoms.
Observation:
- A 64-year-old man presented with subacute memory loss, balance issues, behavioral changes, and seizures.
- MRI revealed hippocampal abnormalities, and PET showed hypometabolism in temporal, insular, and parietal regions.
- Anti-CASPR2 antibodies were detected in serum, confirming the diagnosis of ALE.
Findings:
- Standard treatments including high-dose steroids and immunoglobulins were ineffective.
- Tocilizumab treatment, administered over six months, led to significant clinical and neuroradiological improvement.
- Rituximab was contraindicated due to Hepatitis B core antibody positivity.
Implications:
- Tocilizumab demonstrates efficacy in treating anti-CASPR2 antibody-associated autoimmune limbic encephalitis.
- This case expands therapeutic options for refractory ALE.
- Further research is warranted to explore tocilizumab's role in similar autoimmune neurological conditions.
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