Rasmussen encephalitis (RE) is a rare progressive presumed autoimmune disorder characterized by pharmacoresistant epilepsy and progressive motor and cognitive deterioration. Despite immunomodulation, more than half of the patients with RE ultimately require functional hemispherotomy. In this study, the potential beneficial effects of early initiation of immunomodulation in slowing disease progression and preventing the need for surgical interventions is evaluated.
A retrospective chart review over a 10-year period is conducted at the American University of Beirut Medical Center to identify patients with RE. Five patients with only monthly to weekly seizures at the time of IVIG initiation have favorable outcomes without resorting to surgery, along with a relative preservation of the gray matter volumes in the affected cerebral hemispheres. Motor strength is preserved in those patients, and three are seizure free at their last follow-up visit.
The data in this report suggest that the early initiation of IVIG as soon as a diagnosis of RE is suspected, and particularly before the appearance of motor deficits and intractable seizures, can maximize the beneficial effects of immunomodulation in terms of controlling seizures and reducing the rate of cerebral atrophy. Read the full article here.