[Rasmussen's syndrome, an autoimmune disease].
Rasmussen's disease is an inflammatory, chronic and progressive brain disorder that usually presents with neocortical focal seizures resistant to conventional treatment and culminates in severe deterioration with hemiparesis, cognitive decline and aphasia. Viral infections and antibodies to the GluR3 receptor have been implicated in the physiopathology of this illness and T-cell mediation may play a role in the cerebral inflammatory process. Classical treatment consists of hemispherectomy of various magnitudes depending on cerebral involvement. The association between therapy-resistant epilepsy and autoimmune phenomena due to antibodies against glutamic acid decarboxylase (anti-GAD) have very recently begun to be studied. The discovery of this association led to a new focus and alternative therapies with immunosuppressors, immunoglobulins, steroids and plasmapheresis, alone or in combination, have begun to be tested with variable success. We describe a boy who was diagnosed in the early stages of Rasmussen's syndrome. He tested positive for anti-GAD antibodies and received treatment with immunoglobulins and steroids. After treatment the boy tested negative for anti-GAD antibodies and he remains asymptomatic after ten months.
Carrillo Herranz
A
A
Servicios de Pediatría. Hospital Ramón y Cajal. Madrid. España. acarrillo.hrx@salud.madrid.org
Sánchez Pérez
I
I
Aparicio Meix
J M
JM
Lozano Giménez
C
C
Roy Ariño
G
G
Villar Gimerans
L M
LM
Sánchez Muñoz
L
L
D001323:Autoantibodies / Q000276:immunology*; D002675:Child, Preschool; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis / Q000276:immunology*; D005074:Evoked Potentials, Visual; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D015899:Tomography, Emission-Computed, Single-Photon
D001323:Autoantibodies; D007074:Immunoglobulin G