Rasmussen's encephalitis: Imaging spectrum on simultaneous FDG-PET and MRI imaging correlation
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Published on: March 8, 2013
Murumkar Vivek S1, Karthik Kulanthaivelu1, Chandana Nagaraj1
1Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Insights
Rasmussen encephalitis (RE) is a rare neurodegenerative disease. This study reviews five cases, highlighting typical and atypical imaging findings crucial for early diagnosis and monitoring disease progression.
Area of Science:
- Neurology
- Neuroinflammation
- Pediatric Neurology
Background:
- Rasmussen encephalitis (RE) is a rare, chronic, inflammatory, and neurodegenerative disorder.
- Primarily affecting children, RE presents as drug-refractory epilepsia partialis continua (EPC).
- Immunomodulation may delay progression but rarely improves outcomes.
Observation:
- Imaging, particularly MRI and nuclear imaging, is vital for RE diagnosis and progression monitoring.
- Typical MRI findings include hemispheric and caudate nucleus atrophy, with ventricular and sulcal dilatation.
- This review examines five RE cases, noting one with typical and four with atypical imaging features on PET-MRI.
Findings:
- Atypical imaging presentations of Rasmussen encephalitis can occur, challenging diagnosis.
- PET-MRI revealed diverse imaging patterns in the reviewed cases.
- Early and accurate diagnosis through advanced imaging is critical.
Implications:
- Understanding diverse imaging features aids in earlier and more accurate diagnosis of RE.
- This knowledge can guide treatment strategies and improve patient outcomes.
- Further research into atypical RE presentations is warranted.
Abstract:
Rasmussen encephalitis (RE) is a rare, chronic, idiopathic, progressive, inflammatory, neurodegenerative disease process and typically seen in pediatric cohort. Although primarily a disease affecting children, adult cases with RE have also been reported. It manifests as drug refractory epilepsia partialis continua (EPC). Immunomodulation, although delays progression of disease, seldom influences outcome. Imaging is crucial for early diagnosis, and monitoring disease progression. Magnetic resonance imaging (MRI) is mainstay of imaging with nuclear imaging being a complimentary tool for diagnosing RE. Typical imaging features of RE on MRI are hemispherical atrophy, caudate nucleus atrophy, ex vacuo dilatation of the ventricular system and sulci. We review 5 cases of RE who fulfilled diagnostic criteria proposed by Bien et al. in 2005. One patient had typical imaging pattern of RE while other four patients had atypical imaging features of RE on PET-MRI.
