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Rasmussen encephalitis is a rare unihemispheric inflammatory disease of the brain that leads to intractable seizures, cognitive decline and progressive neurological deficits associated with the affected hemisphere. It predominantly affects children, with the onset in adults having a milder course. Immunotherapy has been suggested to improve the outcome of Rasmussen encephalitis.1,2
In November 2000, a left-handed 37-year-old woman experienced a head trauma with brief loss of consciousness. Shortly after, she had mild clumsiness of her right leg, which went on for the next 2 years. No magnetic resonance imaging (MRI) study was performed at that time. Her family also noticed a change in her character (all of which was retrospectively interpreted as the “prodromal stage” of Rasmussen encephalitis2).
In February 2002, the patient started having epilepsia partialis continua (EPC) of her right hand (later on interpreted as an onset of the “acute stage” of Rasmussen encephalitis2). Apart from EPC and impaired motor function of her right leg and hand (due to the EPC), the neurological examination and electroencephalogram were normal at this time. Cerebrospinal fluid contained 10 cells/μl, had a normal protein level and showed oligoclonal bands. Microbiological studies showed no sign of an infectious agent. MRI of the brain showed a mild left temporal atrophy. …