OBSOLETE: Limbic encephalitis with DPP6 antibodies

Limbic encephalitis with DPP6 antibodies is a rare autoimmune condition that affects the brain, specifically the limbic system, which is the part of the brain involved in memory, emotions, and behavior. In this condition, the body's immune system mistakenly produces antibodies against a protein called DPP6 (dipeptidyl-peptidase-like protein 6), which is found on the surface of nerve cells. These antibodies attack healthy brain tissue, leading to inflammation. Patients typically experience a combination of neurological and psychiatric symptoms. These can include memory problems, confusion, seizures, sleep disturbances, mood changes, and sometimes involuntary movements or tremors. Some patients also develop gastrointestinal symptoms such as diarrhea and weight loss, which can appear before the brain-related symptoms. The disease tends to progress over weeks to months. Note that this entry is marked as 'OBSOLETE' in Orphanet, meaning it may have been reclassified or merged into a broader category of autoimmune encephalitis. Treatment generally involves immunotherapy to suppress the abnormal immune response, including corticosteroids, intravenous immunoglobulin (IVIG), plasma exchange, and sometimes stronger immunosuppressive drugs like rituximab or cyclophosphamide. Early diagnosis and treatment can improve outcomes, though some patients may have a prolonged or relapsing course.

Bring these to your next appointment

• Q1.What type of immunotherapy do you recommend, and what are the potential side effects?,How will you monitor whether the treatment is working?,What are the chances of relapse, and how would a relapse be treated?,Should I be screened for an underlying tumor?,How long will I need to stay on immunosuppressive treatment?,What rehabilitation services might help with my memory and daily functioning?,Are there any clinical trials or new treatments I should know about?

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