ABSTRACT
Rasmussen's encephalopathy (RE) is an uncommon neurological disease of inflammatory origin which is characterized by intractable focal epilepsy, progressive limb weakness, and cognitive deterioration. RE presenting as movement disorder like hemidystonia or hemichorea is a rare occurrence. The duration of prodromal stage of RE is usually in weeks or months. Prolonged prodromal stage like in years is rarely reported. Magnetic resonance imaging (MRI) is a good biomarker in RE and it also suggests the sequential progression of disease. Here we report two cases of RE, one presenting with hemidystonia and other case with unusually prolonged prodromal stage duration of 7 years. In spite of severe hemi-atrophy of brain in second case response to immunomodulators was dramatic.
