Limbic encephalitis with LGI1 antibodies
Alternate names[edit | edit source]
Limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies
Definition[edit | edit source]
Limbic encephalitis with LGI1 antibodies is a relatively new disease characterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation.
Cause[edit | edit source]
The disease occurs when antibodies mistakenly attack LGI1 (leucine-rich glioma inactivated 1), a protein found in the brain that binds together two epilepsy-related proteins called ADAM22 and ADAM23.
Signs and symptoms[edit | edit source]
- This condition ischaracterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation.
- Seizures are common and frequently precede other symptoms.
- About half of people with this diagnosis exhibit a specific type of seizure called faciobrachial dystonic seizures, which resemble brief jerks of the face arm.
- Hyponatremia (low levels of sodium in the blood) may also be present.
Diagnosis[edit | edit source]
- Some people with limbic encephalitis with LGI1 antibodies (5 to 10%) also have a cancer diagnosis (most commonly thymoma and lung cancer), but most do not.
- The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies.
Treatment[edit | edit source]
- If treatment is started when seizures are first noticed, limbic encephalitis may be prevented.
- Treatment is intravenous or oral corticosteroids, intravenous immunoglobulin (IVIG), plasmapheresis (a process where antibodies are removed from the blood), or a combination of these.
Prognosis[edit | edit source]
- Long-term outcome is mostly favorable, although some people are left with some degree of residual impairment.
- Relapse occurs in up to one third of patients.
NIH genetic and rare disease info[edit source]
Limbic encephalitis with LGI1 antibodies is a rare disease.
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