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Good outcome in adult-onset Rasmussen's encephalitis syndrome: is recovery possible? Volume 17, issue 2, June 2015

TEST YOURSELF

1) Is it mandatory to perform brain biopsy for the diagnosis of Rasmussen’s encephalitis (RE)?


(2) What is the main differential diagnosis of adult-onset RE?


(3) What are the most important treatments for adult-onset RE?

 

 

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Answers

(1) No, it is not. The presence of all three part A criteria is sufficient (Bien CG, et al. Neurology 2002b; 58: 250-7).


(2) One should consider all the possible causes of unihemispheric epileptic syndromes, such as malformations, tumours, cortical dysplasia, “epilepsia partialis continua” due to acquired metabolic disorders or genetic metabolic and degenerative progressive neurological disease, inflammatory disease (systemic connective tissue disease, primary CNS angitis, paraneoplastic syndromes), and infectious disease (mainly consider the possibility of HIV infections). Other causes may be proconvulsive drugs and complications of bone marrow transplants.


(3) Surgery is very difficult to propose in adults. The effects of immunological medical treatments have been reported in single cases or small uncontrolled series. Medical treatments include immunomodulatory and immunosuppressant drugs such as steroids, plasmapheresis, immunosuppressive agents (Tacrolimus, Azatioprina), intravenous polyvalent Ig, and monoclonal antibodies (Natalizumab). To prevent the occurrence of severe brain damage, treatments are more useful at the beginning of the disease. As RE is a rare and severe illness, controlled studies are difficult and also unethical, and the report of single cases remains fundamental in order to develop knowledge about this disabling and potentially treatable disease.

 

 

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