Plasma Exchange in Rasmussen's Encephalitis

Abstract

The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. Antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement

“…One of these patients improved transiently after plasma exchange (Rogers et al, 1994). Other reports of temporary or longer lasting improvement of the symptoms of RE by removal of antibodies from the circulation have subsequently been published (Andrews et al, 1996;Palcoux et al, 1997;Antozzi et al, 1998;Granata et al, 2003a). How autoantibodies might lead to brain tissue destruction and seizure activity has been answered in two different ways: Some authors report evidence that GluR3 antibodies mediate an excessive, cytotoxic activation of the glutamate receptor using in vitro (Twyman et al, 1995; and in vivo systems (Levite and Hermelin, 1999).…”

“…Here, treatments with <6 reported patients in the literature will not be discussed further. This leaves the following regimens as the basis for our recommendations: (i) corticosteroids (Chinchilla et al, 1994;Hart et al, 1994b;Granata et al, 2003a); (ii) intravenous immunoglobulins (IVIG) (Walsh, 1991;Hart et al, 1994b;Wise et al, 1996;Leach et al, 1999;Villani et al, 2001;Granata et al, 2003a); (iii) corticosteroids plus IVIG (Hart et al, 1994b;Krauss et al, 1996;Vinjamuri et al, 2000); (iv) plasmapheresis (PEX) or protein A IgG immunoadsorption (PAI) (Andrews et al, 1996;Palcoux et al, 1997;Antozzi et al, 1998;Granata et al, 2003a); and (v) tacrolimus (Bien et al, 2004).…”

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

“…In a case report a patient improved after 6 months of treatment, with stabilization after 42 sessions of PE, and a worsening of the clinical condition during 1 month's interruption . Other reports described only temporary or longer lasting improvement of the symptoms of RE by removal of antibodies from the circulation, but only with long‐term treatments .…”

“…1 month's interruption [5]. Other reports described only temporary or longer lasting improvement of the symptoms of RE by removal of antibodies from the circulation, but only with long-term treatments [6].…”

Medical treatment of Rasmussen's Encephalitis: A systematic review

“…Plasmapheresis reduces the levels of antibody and other immune mediators and complement fractions. The existing data are mainly from case reports with positive initial effect but recurrence on the long-term [21,[49][50][51][52][53]. However, in larger series the rates of seizure response was low 2/13 [40], and 8 others published patients did not respond to plasmapheresis [41,[54][55][56][57][58][59] (Table 3).…”