Abstract
Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.
Highlights
Autoimmune encephalitis is well recognized as a treatable cause of intractable epilepsy
We will be presenting three cases that presented with epilepsy and were all found to subsequently have the respective antibodies known to be associated with a specific autoimmune encephalitis, and we demonstrated symptom control with the use of rituximab
Autoimmune encephalitis testing was negative in the cerebrospinal fluid (CSF), but serum was found positive for N-type anti-voltage-gated calcium channel (VGCC) antibodies, with titer levels at 0.28, obtained via send-out to Mayo Clinic Labs
Summary
Autoimmune encephalitis is well recognized as a treatable cause of intractable epilepsy. Erapy for these disorders include largely some form of immunosuppressants, with steroids, IVIG, or plasma exchange in some combination being the commonly used first line options. Rituximab has been shown to be a promising medication to administer in other autoimmune disorders when the first line immunosuppressive agents fail to control the condition, but its use for autoimmune encephalitis remains unconfirmed. In this case series, we will be presenting three cases that presented with epilepsy and were all found to subsequently have the respective antibodies known to be associated with a specific autoimmune encephalitis, and we demonstrated symptom control with the use of rituximab
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