Limbic encephalitis with LGI1 antibodies

Alternate names

Limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies

Definition

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

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

• 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

• 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

• 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

• 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

• NIH info on Limbic encephalitis with LGI1 antibodies

Limbic encephalitis with LGI1 antibodies is a rare disease.

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