Many people with ovarian teratoma have encephalitis.

On July 2, Dr. Le Quoc Hung, Head of the Department of Tropical Diseases, Head of the Anti-Poison Resuscitation Unit, Cho Ray Hospital, said that in the past 8 months, the number of cases of this disease has skyrocketed, with 4 cases being treated. In Vietnam, not many places have reported this disease, possibly because doctors were unable to diagnose it before, so it is considered a newly discovered disease.

The average age of patients treated at Cho Ray is 23, including 12 women (6 cases with ovarian teratoma) and 5 men (one case with testicular tumor), of which 9 cases had no detected tumor. 82% of patients were admitted to the hospital in very serious condition, comatose, with convulsions.

“If this disease is misdiagnosed as common encephalitis or not detected, the patient will die and the cost of treatment will be high,” said Dr. Hung.

The average cost of treatment for a patient with autoimmune encephalitis is one billion VND. Six patients with ovarian teratoma were operated on by doctors from Cho Ray Hospital and Hung Vuong Hospital. In two cases, both ovaries had to be removed, affecting fertility and endocrine function later on. However, the patients resolved the cause of the problem and lived a normal life from the second month after surgery. Reports from previous hospitals recorded that patients who did not have their tumors removed only recovered their health in the ninth month.

The most serious case was a 16-year-old girl who was diagnosed with an ovarian tumor. She planned to have surgery during the summer vacation, but before she could have surgery, she had a seizure and fell into a coma. To date, after more than 20 plasma exchanges and expensive medications, the cost of treatment for more than a month has been over 1.3 billion VND.

The treatment of this patient required the doctor to “weigh his brain” many times. The doctors discovered tumors on both ovaries, and tried to separate and save the ovaries but were unsuccessful. The hospital also contacted the egg storage facility, but they were unable to store immature eggs, only keeping 8 samples of ovarian tissue. After losing both ovaries, the patient had to take supplemental hormones for the rest of her life.

Autoimmune encephalitis is a condition in which brain parenchyma is damaged by agents that are antibodies produced by the body. Autoimmune encephalitis caused by anti-NMDAR antibodies was first known to the world in 2007. The disease is mainly related to women with ovarian teratoma, with more than 1,500 cases reported as of 2018. As for autoimmune encephalitis caused by many other antibodies, the world has only recorded a few dozen cases so far.

The disease occurs at all ages, mostly in women, and is caused by a virus that attacks nerve cells or tumors (usually ovarian teratoma containing nerve cell tissue) that release NMDA antibodies. These antibodies attack the central nervous system and damage the brain to varying degrees.

According to Dr. Hung, the number of reported cases does not reflect the full extent of the disease because many cases are not diagnosed. However, diagnosing the disease requires a lot of capacity from the medical facility and the qualifications and experience of the doctor.

Several studies worldwide have followed women with NMDAR autoimmune encephalitis, showing that many cases were initially undetected but later developed ovarian teratoma, possibly because the tumor was too small to be diagnosed at first. This causes many patients to have encephalitis relapse after being cured, because the ovarian origin has not been detected and thoroughly treated.

NMDAR autoimmune encephalitis usually begins gradually over three months, with symptoms of forgetfulness, mental changes or psychosis. Patients often have seizures of unknown cause. Of which, the six groups of autoimmune encephalitis symptoms are abnormal behavior or cognitive disorders, language dysfunction, seizures, movement disorders or abnormal movements, perceptual disorders (drowsiness, confusion, coma), autonomic dysfunction and rapid breathing.

To date, the world has not yet reached a complete consensus on the treatment regimen for this disease. The treatment process is long-term and complicated, requiring high-tech interventions such as mechanical ventilation, extracorporeal blood filtration, plasma exchange, and expensive drugs. In some cases, surgery on the tumor (if any) is required to block the source of NMDA antibodies.

“The current issue is whether to screen for NMDA antibodies in women who are found to have ovarian teratoma,” Dr. Hung said, adding that it is better to spend a few million VND on screening tests instead of spending billions on treatment with many risks. The reason is that the disease has a very high mortality rate, almost 100%, and treatment is complicated and expensive if it has become severe.

Dr. Huynh Thi Thuy Mai, Hung Vuong Hospital, said that this is a very new disease in the field of obstetrics and gynecology. Obstetricians are often concerned about whether ovarian tumors are benign or malignant, and not much is known about cases of ovarian teratoma that produce antibodies causing autoimmune encephalitis.

Hung Vuong Hospital examined about 4,000 cases of ovarian teratoma, including 600 surgeries. Doctors usually prescribe regular monitoring when the tumor is small, and only intervene when the tumor is larger than 5 cm or small in size but torsion occurs, and there is suspicion of cancer. Surgery when the tumor is small can cause a decrease in ovarian reserve, affecting the reproductive future as well as the endocrine problems of women.

“These newly discovered cases open up a new research direction in monitoring and treating ovarian teratoma. We will compile statistics, evaluate, and review to have appropriate recommendations and indications for patients,” said Dr. Mai.

Doctors are working together to develop a national standard diagnosis and treatment protocol. People are advised to pay attention to early detection of this disease when relatives change their habits, mental state, have unexplained memory loss, or have involuntary abnormal movements or seizures for no apparent reason.