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Case Reports
. 2023 Oct 7;15(10):e46647.
doi: 10.7759/cureus.46647. eCollection 2023 Oct.

Atypical Rasmussen's Encephalitis

Maria A Alfonso  1 , Martha C Piñeros-Fernández  2   3 , Luisa F Jaimes  4 , Nicolas I Ramos  1
Affiliations
Case Reports

Atypical Rasmussen's Encephalitis

Maria A Alfonso et al. Cureus. .

Abstract

A three-year-old female patient was admitted to our institution due to subacute fever, intermittent vomiting, persistent bilateral mydriasis after cycloplegia, right central facial palsy, and mild right hemiparesis with hyperreflexia. Brain MRI shows encephalitis in frontal, parietal, insular, and left putamen course and loss of cortical volume and white matter of the entire left hemisphere which are features described in Rasmussen's encephalitis (RE). Therapy with intravenous methylprednisolone bolus was initiated, with adequate clinical response. We consider in this case the diagnosis of atypical RE by imaging criteria in the subacute stage. There are few reports of atypical RE without epilepsy or continuous partial epilepsy. Our purpose is to present a case of a patient with RE images without epilepsy seizures and review the diagnostic and therapeutic approach of RE.

Keywords: autoimmune encephalitis; brain magnetic resonance images; case report; encephalitis; focal motor deficit; hemiplegia; language regression; rasmussen syndrome; refractory epilepsy; seizure.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. First brain MRI axial T2
Figure 2
Figure 2. First brain MRI coronal
Figure 3
Figure 3. Second brain MRI with contrast
Decrease of the left hemisphere volume. Note that the frontal, parietal, and temporal lobes are the most affected as observed in the first MRI.
Figure 4
Figure 4. Second brain MRI with contrast axial section
Non-enhancement observed on the left hemisphere.
Figure 5
Figure 5. Second brain MRI, contrasted study, coronal section
This figure details marked compromise of the insular region of left hemisphere also observed in the first brain MRI of the patient.
Figure 6
Figure 6. Normal brain arterial angioresonance
Figure 7
Figure 7. Normal brain venous angioresonance
Figure 8
Figure 8. Normal brain arterial angioresonance coronal section
Figure 9
Figure 9. Normal brain MR spectroscopy of the right hemisphere
Figure 10
Figure 10. Abnormal brain MR spectroscopy of the left hemisphere
Complementary study with brain spectroscopy for hydrogen ions H+, of brain metabolites in short and intermediate echo time (ET) in the region of interest that shows diffuse decrease of all metabolites, without defining the presence of lactate or lipids, which in comparison with the contralateral area, diffuse decrement in neuronal population, and reduction of choline and creatine peaks, which correlates with gliosis changes.
Figure 11
Figure 11. EEG
The  electroencephalographic record was normal
See this image and copyright information in PMC

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