Development from recurrent anti-N-methyl-D-aspartate receptor encephalitis with seizures as the first symptom to autoimmune-associated epilepsy: a case report

TL;DR

A patient with a long history of anti-NMDAR encephalitis developed autoimmune-associated epilepsy, showing how this condition can evolve over time.

Contribution

This case report highlights the progression from anti-NMDAR encephalitis to autoimmune-associated epilepsy over decades.

Findings

The patient had eight episodes of seizures over 30 years, starting with anti-NMDAR encephalitis.

MRI showed hippocampal abnormalities in the last two episodes despite negative antibody tests.

Anti-seizure medicines controlled symptoms better than immunotherapy in later stages.

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a novel autoimmune encephalitis (AE) first identified in 2007. It provides a new direction for clinicians when encountering unexplained symptoms such as seizures, psychotic behavioral abnormalities, speech disorders, and involuntary movements. Most patients have a good prognosis after immunotherapy, but some may experience relapses.

We report a Chinese female patient diagnosed with anti-NMDAR encephalitis. Over the past 30 years, the patient had experienced eight episodes with seizures as the first symptom, which eventually progressed to autoimmune-associated epilepsy. In the last two episodes, both serum and cerebrospinal fluid of the patient were negative for AE-related antibodies, and brain magnetic resonance imaging (MRI) revealed abnormal hyperintensity in the bilateral hippocampi. The patient's symptoms were poorly controlled by immunotherapy but well controlled by anti-seizure medicines.

Patients with a long history of AE and multiple relapses that start with seizures may display alterations of brain structure. Physicians should pay attention to autoimmune-associated epilepsy.

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