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Title: Tumour Removal, Immunotherapy Can Reverse Progress of Newly Recognised Anti-NMDA-Receptor Encephalitis
URL: http://www.pslgroup.com/dg/22E902.htm
Doctor's Guide
October 13, 2008


NEW YORK -- October 13, 2008 -- The clinical features of 100 patients diagnosed with anti-NMDA-receptor encephalitis (ANRE) are reported in a study published early online and in the December issue of The Lancet Neurology.

Josep Dalmau, MD, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, and colleagues discuss how fast tumour removal and immunotherapy are associated with patients' recovery.

"ANRE can … develop without the presence of a tumour. We believe that some of these patients may have a microscopic tumour, but we also acknowledge that most have been followed for many months or years without developing a tumour," the authors write.

"Nevertheless, given that the neurologic disease usually develops before the presence of a tumour is known, all patients with this disease should first be examined for a tumour."

The researchers analysed 100 patients, aged 5 to 76 years, with ANRE, of which 91 were female. All had psychiatric symptoms or memory problems; 76 had seizures; 88 decreased consciousness; 86 involuntary movements frequently involving the face; 69 autonomic nervous system instability; and 66 hypoventilation. Of 98 patients tested for cancer, 58 had tumours, most commonly ovarian teratomas.

Patients who had early tumour treatment along with immunotherapy had better outcomes and fewer neurological relapses compared with the other patients. Of the 100 patients, 75 recovered or had mild neurological deficits, while 25 had severe deficits or died.

Patients' improvement was associated with a decrease in anti-NMDA receptor antibodies. The researchers conclude that these antibodies reduced the numbers of cell-surface NMDA receptors and receptor clusters in the postsynaptic dendrites of the nervous system; this effect could be reversed by removing the antibodies.

"A well-defined set of clinical characteristics is associated with anti-NMDA-receptor encephalitis. The pathogenesis of the disorder seems to be mediated by antibodies," the authors write.

In an accompanying comment, Angela Vincent, Department of Clinical Neurology, University of Oxford, Oxford, United Kingdom, and Christian Bien, MD, University of Bonn, Bonn, Germany, write: "The clinical presentation of psychosis-like symptoms, seizures, abnormal movements, and autonomic disturbances is highly characteristic of NMDA-receptor antibodies in both female and male patients."

"This syndrome has already become an essential consideration in the diagnosis of subacute or acute encephalopathies, particularly in young people, and one should expect the clinical phenotype to expand further as more cases are reported. This is certainly an important development in the study of autoimmune encephalopathies."

SOURCE: The Lancet Neurology

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