A case of Rasmussen encephalitis treated with rituximab.
BACKGROUND
A 20-year-old woman was referred to our intensive care unit with a high frequency (every 1-2 min) of focal motor seizures. She had been diagnosed as having biopsy-proven Rasmussen encephalitis (RE) of the right hemisphere 7 years previously, since when she had been treated with numerous antiepileptic drugs, as well as with continuous immunotherapies, such as tacrolimus, corticosteroids, mycophenolate mofetil, intravenous immunoglobulin and immunoadsorption. Although hemispherectomy had been avoided due to slow progression of RE, she had not been seizure-free for more than 7 weeks since diagnosis.


METHODS
EEG and MRI.


METHODS
Focal motor status epilepticus associated with right hemispheric RE, causing continuous epileptic activity and unilateral atrophy with edema in the right hemisphere.


RESULTS
Immunoadsorption was used initially to treat the seizures. Once they had ceased, we used 375 mg/m2 intravenous rituximab--a monoclonal anti-CD20 antibody--once-weekly for 4 weeks to stabilize the condition, leading to complete depletion of CD19+ B cells. Rituximab infusions were used again when concentrations of CD19+ B cells rose and focal seizures re-emerged. The patient remained on antiepileptic therapy (levetiracetam, oxcarbazepine, zonisamide and phenobarbital) throughout treatment.
Thilo
Barbara
B
Department of Neurology, Christianalbrechts-University, Kiel, Germany. b.thilo@neurologie.uni-kiel.de
Stingele
Robert
R
Knudsen
Karina
K
Boor
Rainer
R
Bien
Christian G
CG
Deuschl
Günther
G
Lang
Nicolas
N
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19 / Q000276:immunology / Q000378:metabolism; D018951:Antigens, CD20 / Q000627:therapeutic use; D016175:B-Lymphocyte Subsets / Q000187:drug effects / Q000276:immunology / Q000378:metabolism; D004660:Encephalitis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D000069283:Rituximab; D013226:Status Epilepticus / Q000150:complications* / Q000473:pathology; D016896:Treatment Outcome; D055815:Young Adult
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D018951:Antigens, CD20; D007155:Immunologic Factors; D000069283:Rituximab