Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement.
Rasmussen's syndrome is a chronic encephalitis characterized by intractable focal epilepsy and progressive neurologic deterioration with lateralized brain destruction. In the early stages of the disease, the diagnosis can be difficult to make, and brain biopsy is often performed. We evaluated the patterns of cerebral glucose metabolism using 2-deoxy-2-[18F]-fluoro-D-glucose positron emission tomography (PET) in 15 children (age range 2.9-15.4 years, mean age 8.7 +/- 4.3 years) with Rasmussen's syndrome. In 6 patients evaluated early (< or = 1 year of onset of seizures), the PET scan showed areas of abnormal metabolism restricted mostly to the frontal and temporal regions, whereas the posterior cortex was preserved. Pathologic changes seen in the resected cortex were more pronounced in cortical areas of abnormal metabolism than in regions showing normal metabolism. In 9 patients evaluated later (>1 year after onset of seizures), the PET scan showed more diffuse hemispheric metabolic abnormalities including the occipital cortex, but the abnormalities remained highly lateralized. These patterns of glucose metabolic abnormalities in the early and late stages of the disease may facilitate the diagnosis of Rasmussen's syndrome and assist guidance of biopsy in early cases, when structural neuroimaging is still normal.