Limbic encephalitis in a patient with systemic lupus erythematosus successfully treated with high-dose glucocorticoids and intravenous cyclophosphamide therapy: the potential pathogenicity of anti-glutamate receptor antibodies

Abstract

Limbic encephalitis (LE) is a clinically defined syndrome characterised by an acute or subacute impairment of short-term memory, seizures and psychiatric symptoms (i.e. depression, anxiety and hallucination). LE could come from certain conditions where the neuropsychiatric systemic lupus erythematosus (NPSLE) of the multiple central nervous system is layered. In this report, we describe a 46-year-old Japanese female with SLE that suddenly presented with seizures, sensory aphasia and pseudobulbar affect. She was diagnosed with severe NPSLE presenting clinical LE (LE-SLE) by excluding malignancies, infectious encephalitis and symptomatic epilepsy using diffusion-weighted magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT). The patient showed a rapid response to treatment with methylprednisolone pulses followed by high-dose prednisolone and intravenous cyclophosphamide. She had elevated anti-glutamate receptor antibodies (anti-GluRs) in her serum and cerebrospinal fluid (CSF) on admission, and the titres decreased to a normal range at a one-year follow up. Our case highlights the importance of measuring anti-neuron antibodies including anti-GluRs in NPSLE patients, and suggests that the reduction of these pathogenic autoantibodies in serum or CSF could be a prognostic marker.

Keywords:

• Systemic lupus erythematosus
• neuropsychiatric systemic lupus erythematosus
• Limbic encephalitis
• Anti-glutamate receptor antibodies
• Intravenous cyclophosphamide therapy

Patient consent

The patient provided written informed consent for publication of her data.

Ethical statement

Not applicable.

Conflict of interest

H. Shoda received fees from Pfizer, Bristol-Myers Squibb, Eli Lilly, GlaxoSmithKline, Daiichi Sankyo, Gilead, Janssen, Sanofi, AbbVie, Chugai, Takeda, Asahi Kasei, Astellas, and Daiichi-Sankyo. Y. Takahashi received academic donation for research of immune-mediated epilepsy from Eizai co. Ltd. K. Fujio received speaking fees, and/or honoraria from Tanabe Mitsubishi, Bristol Myers, Eli Lilly, Chugai, Jansen, Pfizer, Ono, AbbVie, Ayumi, Astellas, Sanofi, Novartis, Daiichi Sankyo, Eisai, Asahi Kasei, Japan Blood Products Organisation, and Kowa, and received research grants from Tanabe Mitsubishi, Bristol Myers, Eli Lilly, Chugai, AbbVie, Ayumi, Astellas, Sanofi, Eisai, Tsumura & Co., and Asahi Kasei. All the other authors declare no competing financial interests.