20-year-old male with progressive status epilepticus

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20-year-old male with progressive status epilepticus Teaching NeuroImages Neurology Resident and Fellow Section

Vignette Onset at age 15 with a single generalized tonic-clonic seizure. After 2 years seizure-free, patient experienced emergence of dyscognitive and hypermotor seizures. In the most recent 18 months, patient showed progressive pattern of complex- and simple-partial status epilepticus involving left extremities. Leiva-Salinas et al

Imaging Leiva-Salinas et al Coronal T1-weighted MRI from 2013 (A) and 2015 (B), show progressive cerebral atrophy, right more than left, most prominent in the striatum (arrows). Initial MRI from 2011 was normal. FDG-PET shows right hemispheric hypometabolism predominantly involving the striatum (C), and frontal and temporal lobes (blue regions, D). Leiva-Salinas et al

20-year-old male with progressive status epilepticus Diagnosis: Rasmussen Encephalitis. The characteristic findings of Rasmussen encephalitis are diffuse unilateral cerebral hypometabolism on FDG-PET with corresponding regions of brain atrophy on MRI, which is often progressive. FDG PET imaging may increase the diagnostic confidence in cases in which the MRI findings are subtle. 1. Villani F, Pincherle A, Antozzi C, et al. Adult-Onset Rasmussen’s Encephalitis: Anatomical-Electrographic-Clinical Features of 7 Italian Cases. Epilepsia 2006,47(Suppl 5):41–46. 2. Leach JP, Chadwick DW, Miles JB, Hart IK. Improvement in adult-onset Rasmussen’s encephalitis with long-term immunomodulatory therapy. Neurology 1999;52:738–742. Leiva-Salinas et al