Late onset Rasmussen encephalitis: complete remission after one session of plasmapheresis

Rasmussen’s encephalitis (RE) is an immunomediated disorder characterized by unilateral hemispheric atrophy, drugresistant focal epilepsy and progressive neurological deficits [1]. The etiology of RE is unknown. In the adult form the semiology may include motor signs, visual, olfactory and dysmnesic features, as well as loss of contact [2]. The outcome of RE is disappointing. Treatment includes corticosteroids, immunoglobulins, plasma exchange (PE), cyclophosphamide and tacrolimus but also antiepileptic drugs (AEDs) and, in the most severe cases, functional hemispherectomy or hemispherotomy [3,4]. We describe a case of late-onset RE with an excellent outcome after one PE cycle.

[1]  G. Defer,et al.  Le syndrome de Rasmussen à début tardif : caractéristiques cliniques et thérapeutiques , 2007 .

[2]  C. Elger,et al.  Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. , 2005, Brain : a journal of neurology.

[3]  G. Broggi,et al.  Experience with immunomodulatory treatments in Rasmussen’s encephalitis , 2003, Neurology.

[4]  R. Spreafico,et al.  Long-term selective IgG immunoadsorption improves Rasmussen's encephalitis , 1998, Journal of Neuroimmunology.

[5]  M. Tardieu,et al.  Plasma exchange in Rasmussen's encephalitis. , 1997, Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis.

[6]  J. McNamara,et al.  Plasmapheresis in Rasmussen's encephalitis , 1996, Neurology.

[7]  M. Baulac,et al.  [Late onset Rasmussen's syndrome: clinical and therapeutic characteristics]. , 2007, Revue neurologique.