Abstract
Anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) has been classified as an autoimmune LE with a subacute course. Many patients with anti-LGI1 LE have normal or minimal cerebrospinal fluid (CSF) findings. Cerebrospinal fluid 14-3-3 protein or neuron specific enolase is usually seen in Creutzfeldt-Jakob disease (CJD) with high sensitivities, but can also be positive in other paraneoplastic and autoimmune encephalitides, which can make diagnosis challenging. The mainstay of treatment for anti-LGI1 LE generally focuses on steroids, intravenous immunoglobulin (IVIG), plasmapheresis, and/or rituximab. All the aforementioned modalities can be used in the treatment of anti-LGI1 LE and since this condition is highly responsive to treatment with steroids, prompt diagnosis can help stall the progression of this disease. Here, we present a case of anti-LGI1 LE that initially improved with empiric immunotherapy and showed definitive return to baseline with initiation of rituximab.
Highlights
All the aforementioned modalities can be used in the treatment of anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) and since this condition is highly responsive to treatment with steroids, prompt diagnosis can help stall the progression of this disease
Anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) is a subacute disorder characterized by faciobrachial dystonic seizures (FBDS), temporal lobe epilepsy, prominent short-term memory deficits, altered mental status (AMS) [1], and antibodies against the LGI1 subunit of the voltage-gated potassium channel (VGKC) complex on neurons [2]
We report a case presenting with FBDS, progressive cognitive and memory decline, initially negative magnetic resonance imaging (MRI) and electroencephalogram (EEG) findings, and positive cerebrospinal fluid (CSF) protein 14-3-3 that dramatically improved with empiric treatment with immunotherapy as well as initiation of rituximab therapy
Summary
Anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) is a subacute disorder characterized by faciobrachial dystonic seizures (FBDS), temporal lobe epilepsy, prominent short-term memory deficits, altered mental status (AMS) [1], and antibodies against the LGI1 subunit of the voltage-gated potassium channel (VGKC) complex on neurons [2]. Repeat CSF studies done during this admission including an autoimmune profile, West Nile virus, herpes simplex virus 1 (HSV 1) and HSV 2, immunofixation electrophoresis, neuron specific enolase, rheumatoid factor (RF), antinuclear antibody (ANA) comprehensive panel, erythrocyte sedimentation rate (ESR), rapid plasma reagin (RPR) and venereal disease research laboratory (VDRL), Vitamin D, anti-neutrophil cytoplasmic antibody (ANCA) vasculitides antibodies, paraneoplastic profile of the serum and CSF all came back within normal limits During this time the patient had an increased appetite with fixation on sweet products, decreased short-term memory, ataxia, mild postural kinetic tremor bilaterally and exhibited obsessive behaviors such as continuously picking at her teeth and fingers. The patient showed marked improvements on steroids, with resolution of faciobrachial seizures on day 5 of this second admission (DOI #31) and improvement of mental function She returned near baseline, with some passive lethargic behavior persisting. At another outpatient follow-up, the patient and family endorsed dramatic improvement of cognitive function after the start of rituximab therapy and no continued AMS experiences
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