AUGUST NEWSLETTER

Selected Highlighted News in the field of Autoimmune

Encephalitis  AUGUST 2018 2nd edition

Dr. Dalmau Podcast

This is a 10-minute podcast of Dr. Josep Dalmau discussing how he began his work in autoimmune encephalitis and his research in HSV encephalitis that can become anti-NMDAr encephalitis a few weeks after the HSV encephalitis. He reports that this now occurs in 27% of cases (up from the 20% he reported in the past) and explains that these cases are still under diagnosed.

It is much easier to diagnose cases age 4 and under due to the specific movement disorder, but older patients are frequently misdiagnosed because the clinician continues to look for a connection with the current disease (which is anti-NMDAr encephalitis, but the doctor has not realized it yet because they are unfamiliar with it) and the original HSV encephalitis.  So, it is important for doctors to know about this ever more common occurrence.

How are lumbar puncture headaches prevented and treated?

Up to 30 percent of patients experience a headache after a lumbar puncture (also known as a spinal tap).  The headache, which is typically caused by spinal fluid that leaks out when the needle is inserted, is usually dull and throbbing and gets worse when you sit up or stand. Pain can range from mild to incapacitating and may be accompanied by dizziness, ringing in the ears, blurred or double vision, nausea, and neck stiffness.

Treatment for anti-NMDAr encephalitis

Best-practice care is still being established in patients with anti-NMDA receptor encephalitis. Expert opinion provides increasingly clear guidelines for treating the auto-antibody and immune response. Early recognition is very important because outcomes are best in patients treated early in the course of disease. After diagnosis, treatment focuses on immunotherapy and appropriate treatment of a tumor if it exists. Corticosteriods and intravenous immunoglobulin (IVIg) or plasma exchange are recommended in managing the immune response; these therapies appear to work best in the scenario where an underlying tumor has been removed. The use of plasma exchange is challenging in agitated patients or cases with autonomic instability, and IVIg is often preferred. In patients without an underlying tumor, first line immunotherapy is often not sufficient, and treatment with rituximab or cyclophosphamide may be required. Current recommendations suggest using either or both second line immunotherapies if no improvement is observed with corticosteroids and IVIg. In patients without a tumor (in whom relapse is more common), continued immunosuppresion with mycophenolate mofetil or azathioprine is recommended for at least 1 year and periodic screening for an ovarian teratoma over 2 years.