Thursday, March 6, 2014

In the January 17, 2014, early view of the journal, NeurologyDoctor Viaccoz presents an observational study of 71 adult patients diagnosed with NMDA receptor antibody encephalitis (an inflammation of the brain) at the French Pyrenees Neoplastic Neurological Syndrome Reference Center. This is an important study because little is known about this encephalitis that causes the sudden onset of difficult to control seizures, is recently described, and is being more recognized by clinicians throughout the world.

  • The study looked at 13 adult male patients, the third largest series on the encephalitis and the observation was that 8 of 13 adult men, or 61%, more often presented with seizures than adult women.

  • Women tend to present with abnormal behavior and psychiatric symptoms.

  • The initial seizures in men were frequently partial, 5 of the 8; while in women they were usually generalized, 1 to 58 had partial seizures.

  • All patients, regardless of gender, subsequently progressed to develop a classic picture of anti-NMDA receptor encephalitis.

  • At the peak of the disease, more than 50% of the patients had behavioral and psychiatric features, seizures, cognitive dysfunction consisting of amnesia, speech disorder, alteration of mental status, movement disorders, and fluctuating level of consciousness.

  • An underlying tumor was identified in 41% of women, all of which were ovarian teratomas; none were found in men.

  • Most of the 71 patients were treated with steroids and IV immunoglobulin, and, if these treatments failed, the most common second line therapy was rituximab.

  • A year after onset of disease, 80% had substantially improved; one man (8%) died from sepsis, and, in women, the mortality rate was 5%.

  • The authors also suggest that hormonal factors may serve as a possible explanation for the different sex-related symptom presentation.

Authored by: Joseph I. Sirven MD on 3/2014