Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report

Rasmussen's encephalitis (RE) is a rare, chronic, and progressive inflammatory brain disorder, characterized by drug-resistant seizures, cognitive decline, and unihemispheric atrophy. Focal neurological deficits and focal motor seizures are typically the initial manifestations. However, we enco...

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Main Authors: Rihab Ben Dhia, MD, Yasmine Saad, MD, Narjes Gouta, MD, Mariem Mhiri, MD, Mahbouba Frih-Ayed, PhD
Format: Article
Language:English
Published: Elsevier 2025-07-01
Series:Radiology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S1930043325002882
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author Rihab Ben Dhia, MD
Yasmine Saad, MD
Narjes Gouta, MD
Mariem Mhiri, MD
Mahbouba Frih-Ayed, PhD
author_facet Rihab Ben Dhia, MD
Yasmine Saad, MD
Narjes Gouta, MD
Mariem Mhiri, MD
Mahbouba Frih-Ayed, PhD
author_sort Rihab Ben Dhia, MD
collection DOAJ
description Rasmussen's encephalitis (RE) is a rare, chronic, and progressive inflammatory brain disorder, characterized by drug-resistant seizures, cognitive decline, and unihemispheric atrophy. Focal neurological deficits and focal motor seizures are typically the initial manifestations. However, we encountered an exceptionally rare case where occipital seizures were the sole presenting feature. This unusual presentation poses unique diagnostic and therapeutic challenges, stemming from its posterior seizure onset and atypical clinical profile, complicating recognition and effective management. We report the case of an 11-year-old boy with no prior medical or familial history of epilepsy. Born to nonconsanguineous parents, his prenatal, perinatal, and early developmental milestones were unremarkable. The patient exhibited normal psychomotor development until 5 years prior to presentation, when occipital drug-resistant visual seizures began. Clinical, imaging, and electrophysiological findings revealed posterior-onset seizures and unilateral hemispheric atrophy consistent with Rasmussen's encephalitis. Posterior-Onset seizures can present as the exclusive initial manifestation of Rasmussen's encephalitis. This case highlights the importance of considering this diagnosis in patients with newly diagnosed drug-resistant visual seizures particularly when accompanied by cognitive decline.
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spelling doaj-art-3cccfbbbe5c94bea9bf4de8f6c807c472025-08-20T01:50:01ZengElsevierRadiology Case Reports1930-04332025-07-012073161316610.1016/j.radcr.2025.03.078Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case reportRihab Ben Dhia, MD0Yasmine Saad, MD1Narjes Gouta, MD2Mariem Mhiri, MD3Mahbouba Frih-Ayed, PhD4Department of Neurology, Fattouma Bourguiba University Hospital, Monastir, TunisiaCorresponding author.; Department of Neurology, Fattouma Bourguiba University Hospital, Monastir, TunisiaDepartment of Neurology, Fattouma Bourguiba University Hospital, Monastir, TunisiaDepartment of Neurology, Fattouma Bourguiba University Hospital, Monastir, TunisiaDepartment of Neurology, Fattouma Bourguiba University Hospital, Monastir, TunisiaRasmussen's encephalitis (RE) is a rare, chronic, and progressive inflammatory brain disorder, characterized by drug-resistant seizures, cognitive decline, and unihemispheric atrophy. Focal neurological deficits and focal motor seizures are typically the initial manifestations. However, we encountered an exceptionally rare case where occipital seizures were the sole presenting feature. This unusual presentation poses unique diagnostic and therapeutic challenges, stemming from its posterior seizure onset and atypical clinical profile, complicating recognition and effective management. We report the case of an 11-year-old boy with no prior medical or familial history of epilepsy. Born to nonconsanguineous parents, his prenatal, perinatal, and early developmental milestones were unremarkable. The patient exhibited normal psychomotor development until 5 years prior to presentation, when occipital drug-resistant visual seizures began. Clinical, imaging, and electrophysiological findings revealed posterior-onset seizures and unilateral hemispheric atrophy consistent with Rasmussen's encephalitis. Posterior-Onset seizures can present as the exclusive initial manifestation of Rasmussen's encephalitis. This case highlights the importance of considering this diagnosis in patients with newly diagnosed drug-resistant visual seizures particularly when accompanied by cognitive decline.http://www.sciencedirect.com/science/article/pii/S1930043325002882Rasmussen encephalitisAtypical Rasmussen encephalitisVisual seizureEncephalopathy
spellingShingle Rihab Ben Dhia, MD
Yasmine Saad, MD
Narjes Gouta, MD
Mariem Mhiri, MD
Mahbouba Frih-Ayed, PhD
Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
Radiology Case Reports
Rasmussen encephalitis
Atypical Rasmussen encephalitis
Visual seizure
Encephalopathy
title Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
title_full Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
title_fullStr Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
title_full_unstemmed Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
title_short Diagnostic misdirection in posterior-onset Rasmussen's encephalitis: A case report
title_sort diagnostic misdirection in posterior onset rasmussen s encephalitis a case report
topic Rasmussen encephalitis
Atypical Rasmussen encephalitis
Visual seizure
Encephalopathy
url http://www.sciencedirect.com/science/article/pii/S1930043325002882
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