As research on autoimmune encephalitis has progressed over the past 10 years, pioneers in the field have often referred to it as a heterogeneous group of disorders. The commonality in various forms of AE is the underlying pathologic mechanism in a patient’s own immune system. For unknown reason the patient’s immune system has produced antibodies that are able to cross into the brain and cause disruption of synaptic function in neurons. (It is thought these antibodies are sometimes produced after a recent infection.) Depending on what parts of the brain are affected, various symptoms can result including the following: loss of cognitive function, memory lapses, involuntary movements, seizures, odd sensory experiences, problems with speech, appetite or behavioral disturbances, vision problems including hallucinations, insomnia, and other neuropsychiatric symptoms.
Doctors and researchers have discovered ~22 antibodies thus far. One form of autoimmune encephalitis is caused by the anti-LGI1 antibody, and can result in frequent brief movements or twitches of one side of the face and a limb on the same side. These movements are “faciobrachial dystonic seizures” or sometimes referred to as “focal dystonia” as there is not always an abnormality on EEG. One such case was reported in the Journal of the American Medical Association Neurology recently. In this case a gentleman experienced difficulty in finding words, and short-term memory loss over several months. In addition he had a rising sensation in his abdomen that went to his chest and head, along with “electric-like jolts” and speech difficulty. It was reported that his EEG did not show any abnormality correlating with the involuntary movements, however his MRI showed abnormalities in the temporal lobes and basal ganglia. He had further indications on his PET scan that allowed physicians to diagnose his disease, along with positive antibodies to LGI1, which is a secreted protein. Thankfully aggressive immunotherapy resulted in an improvement in this case. To read this article in the “Clinical Review & Education” section of this issue of JAMA Neurology, please click here.
A special thank you to Jack Grant for sharing his story with AEA in hope of aiding others.
Post created by Lynn Chapman