Ovarian Dermoids and Autoimmune Encephalitis: A Zebra to Keep in Mind

Andrew M. Kaunitz, MD


December 14, 2018

Hello. I am Andrew Kaunitz, professor and interim chair in the Department of Obstetrics and Gynecology at the University of Florida College of Medicine in Jacksonville. Today I'd like to discuss "Ovarian Dermoids and Autoimmune Encephalitis: A Zebra to Keep in Mind."

Some years ago, I read with interest an editorial[1] in which Dr Robert Barbieri, chair, and Dr Rachel Clark, gynecology-oncology fellow, both at Brigham and Women's Hospital in Boston, described a patient in whom a dermoid cyst of the ovary caused encephalitis.

Recently, a column in The New York Times Magazine,[2] which detailed a similar case, reminded me of the importance of keeping this rare but highly treatable condition on our radar screens.

Both cases involved a young woman presenting with severe psychiatric and neurologic symptoms and signs. Ultimately, pelvic imaging suggested the presence of a benign cystic teratoma (or dermoid), and laboratory assessment revealed anti-N-methyl-D-aspartate (anti-NDMA)-receptor antibodies.

According to D. Josep Dalmau, a neurologist at the University of Pennsylvania who, along with colleagues, has published extensively in this area, patients with autoimmune encephalitis associated with anti-NDMA antibodies may present with prodromal headache and fever followed in a few days by an array of additional symptoms, including anxiety, agitation, bizarre behavior, hallucinations, delusions, disorganized thinking, insomnia, memory deficits, seizure activity, stupor resembling catatonia, abnormal movements, autonomic instability, and even hypoventilation requiring intubation with mechanical ventilation.[3,4]

A brain MRI is often abnormal in patients with autoimmune encephalitis, and cerebrospinal fluid analysis reveals an elevated white blood cell count in most cases.

Among women over 18 years of age with anti-NMDA receptor encephalitis, approximately 50% are ultimately found to have ovarian dermoids.[5] Accordingly, pelvic imaging should be part of the evaluation of any female patient with symptoms suggesting autoimmune encephalitis.

Fortunately, removal of the dermoid tumor, which reduces the titer of anti-NDMA antibodies, improves symptoms in most cases of encephalitis caused by this condition. Although benign cystic teratomas are common, fortunately it is rare that these tumors cause encephalitis.

Given their presenting symptoms, women with autoimmune encephalitis are characteristically under the care of neurologists or psychiatrists. By being aware of this rare condition and collaborating with colleagues in other specialties, obstetrician-gynecologists and other women's health clinicians can improve the outcomes of women with this highly treatable form of encephalitis.

Thank you for the honor of your time, and happy holidays. I am Andrew Kaunitz.


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