Vijay M Ravindra1,2, Marcus D Mazur1,2, Carrie A Mohila3, Matthew T Sweney4,5, Aimee Hersh6,7, Robert J Bollo8,9. 1. Department of Neurosurgery, University of Utah School of Medicine, Salt Lake City, UT, USA. 2. Division of Pediatric Neurosurgery, Primary Children's Hospital, 100 North Mario Capecchi Drive, Suite 1475, Salt Lake City, UT, 84113, USA. 3. Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA. 4. Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA. 5. Division of Pediatric Neurology, Primary Children's Hospital, Salt Lake City, UT, USA. 6. Department of Rheumatology/Immunology, University of Utah School of Medicine, Salt Lake City, UT, USA. 7. Division of Pediatric Rheumatology/Immunology, Primary Children's Hospital, Salt Lake City, UT, USA. 8. Department of Neurosurgery, University of Utah School of Medicine, Salt Lake City, UT, USA. robert.bollo@hsc.utah.edu. 9. Division of Pediatric Neurosurgery, Primary Children's Hospital, 100 North Mario Capecchi Drive, Suite 1475, Salt Lake City, UT, 84113, USA. robert.bollo@hsc.utah.edu.
Abstract
PURPOSE: Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of Rasmussen encephalitis. CASE REPORT: We report the case of a 12-year-old girl with a history of cognitive decline and right arm weakness. Magnetic resonance imaging demonstrated diffuse left hemispheric cortical and subcortical atrophy suggestive of Rasmussen encephalitis. The patient had no clinical history of seizures, and electroencephalography did not demonstrate epileptiform abnormalities. Craniotomy for open brain biopsy was performed, and histopathologic evaluation identified Rasmussen encephalitis with cortical dysplasia (dual pathology). CONCLUSIONS: To the best of our knowledge, this is the third case of Rasmussen encephalitis diagnosed by both imaging and histopathology that had no clinical or electroencephalographic evidence of seizures and is the only case of Rasmussen encephalitis with cortical dysplasia without epilepsy.
PURPOSE:Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of Rasmussen encephalitis. CASE REPORT: We report the case of a 12-year-old girl with a history of cognitive decline and right arm weakness. Magnetic resonance imaging demonstrated diffuse left hemispheric cortical and subcortical atrophy suggestive of Rasmussen encephalitis. The patient had no clinical history of seizures, and electroencephalography did not demonstrate epileptiform abnormalities. Craniotomy for open brain biopsy was performed, and histopathologic evaluation identified Rasmussen encephalitis with cortical dysplasia (dual pathology). CONCLUSIONS: To the best of our knowledge, this is the third case of Rasmussen encephalitis diagnosed by both imaging and histopathology that had no clinical or electroencephalographic evidence of seizures and is the only case of Rasmussen encephalitis with cortical dysplasia without epilepsy.
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