Tuesday, March 12, 2013

Encephalitis associated with ovarian tumors


It is one thing to learn something new every day, but another to come across something you have never heard of.

Enter anti-NMDAR encephalitis secondary to ovarian teratomas.

Anti-NMDAR encephalitis syndrome is a constellation of psychiatric symptoms, memory changes, altered level of consciousness, and/or central hypoventilation due to the interaction of antibodies to NMDAR (receptor in brain tissue) with the hippocampus.

This condition can present at any age group, and can be associated with teratomas (in 50% of females above 12 years of age with anti NMDAR encephalitis); mainly the ovarian type.  It is the second most common type of autoimmune encephalitis, and is believed to be the result of interaction of auto antibodies to NMDAR (generated in response to ectopic neuronal tissue in the teratoma) with the hippocampus.  The diagnosis is confirmed with anti-NMDAR antibodies in the blood or CSF.

The treatment for this condition consists of immune therapy (steroids/plasmapharesis/IV immunoglobulin), tumor resection, and possible need for immune suppression.
80% of patient have substantial improvement of symptoms within 24 months of initiation of therapy.  During this time, the risk of relapse is 12%.

News to me.

Reference:

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