What is LGI1 encephalitis?
LGI1 LE is an autoimmune encephalitis whose clinical manifestations are memory deterioration, FBDS, epileptic seizure, mental disorders, and hyponatremia. Brain MRI shows that this autoimmune disease mainly involves the mediotemporal lobe and the hippocampus.
What are Faciobrachial dystonic seizures?
Faciobrachial Dystonic Seizure (FBDS) is a rare form of epilepsy characterized by frequent brief seizures, which primarily affect the arm and face. It has been described as the pathognomonic semiology for autoimmune limbic encephalitis (ALE) [1].
What is Dppx antibody?
DPPX antibodies are predominantly IgG1 and IgG4 and associate with cognitive-mental deficits and symptoms of CNS hyperexcitability that are usually preceded by diarrhea, other gastrointestinal symptoms, and weight loss.
Can a person with LGI1 antibodies get lung cancer?
[1] [5] [6] 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. [1] [6] The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies.
How are LGI1 antibodies related to epilepsy?
[1] [2] [3] [4] [5] [6] Hyponatremia (low levels of sodium in the blood) may also be present. [1] [3] [6] 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.
Can a LGI1 antibody cause rapid progressive dementia?
This case raises the awareness that a rapid progressive dementia with predominant memory deficits could be induced by immunoreactions against LGI1. The better recognition will be great importance for the early diagnosis, essential treatment, even a better prognosis.
Is there an antibody test for LGI1 encephalitis?
LGI1 antibody encephalitis may easily be mistaken for other neuro-psychiatric syndromes, and clinical identification of the syndrome and confirmation with antibody testing is paramount, as EEG, MRI, and routine lumbar puncture studies may be unremarkable, particularly early in the clinical course.
