New Onset British Accent, Acute Behavioral Changes, and Seizures: A Unique Presentation of NMDAR Encephalitis

Abstract EN

The leading cause of autoimmune encephalitis is N-methyl-D-aspartate receptor (NMDAR) encephalitis.

Symptoms can present as prominent behavioral abnormalities prompting inaccurate psychiatric diagnoses.

Psychiatric features such as bizarre behavior, agitation, anxiety, delusions, and hallucinations are well noted in the current literature, but a manifestation of foreign accent syndrome has, to our knowledge, never been reported in cases of encephalitis.

Once diagnosed, initiation of therapy can result in effective treatment.

Here, we present a case of a 32-year-old female with new onset seizures and marked behavioral changes, such as speaking in a foreign accent, who was empirically treated for NMDAR encephalitis due to strong clinical suspicion, showed no improvement with first line therapy with IVIG and IV steroids, and finally had rapid resolution of symptoms with the early initiation of second line therapy of rituximab.

In a young female presenting with nonspecific behavioral changes, NMDAR encephalitis should be on the differential and, although CSF antibodies are definitively diagnostic, there should be a low threshold to start empiric therapy and escalate to second line treatment.