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|Year : 2021 | Volume
| Issue : 4 | Page : 1140-1141
Rasmussen Encephalitis with Ipsilateral Brain Stem and Contralateral Cerebellar Atrophy
Ankita Agarwal1, Kanwaljeet Garg2, Aishwarya Gulati1, Parveen Gulati1
1 MRI Division, Dr. Gulati Imaging Institute, New Delhi, India
2 Department of Neurosurgery, All India Institutes of Medical Sciences, New Delhi, India
|Date of Submission||29-Jun-2018|
|Date of Decision||10-Apr-2020|
|Date of Acceptance||15-May-2021|
|Date of Web Publication||2-Sep-2021|
J – 16, Hauz Khas Enclave, New Delhi - 110 016
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal A, Garg K, Gulati A, Gulati P. Rasmussen Encephalitis with Ipsilateral Brain Stem and Contralateral Cerebellar Atrophy. Neurol India 2021;69:1140-1
Rasmussen encephalitis is an uncommon chronic inflammatory disease of the central nervous system usually presenting in the pediatric population with a mean age of 6–8 years, having classical symptoms of intractable seizures, neurodegeneration, cognitive decline, hemiparesis, or hemiplegia. Adult onset of disease is seen only in ten percent of the cases. The disease is slowly progressive in nature. Clinical history, magnetic resonance imaging (MRI) findings, and electroencephalogram (EEG) correlation are almost diagnostic of this condition and preclude the need for brain biopsy, which is an invasive procedure. The condition was first described by Rasmussen et al.
We report a case of a 26-year-old male patient presenting with recurrent seizures starting at the age of eight years, with no other relevant history. After a year, the patient developed weakness of the right half of his body followed by cognitive decline with frequent violent behavior. The patient continued to have poorly controlled, intractable seizures, thus a surgical treatment was being planned. MRI performed on a 3.0 T system revealed marked unilateral cerebral atrophy on the left side, apparent as loss of volume with dilated sulcal and cisternal spaces, along with atrophy of the basal ganglia. There was ex vacuo dilatation of left lateral and third ventricles. The underlying brain showed diffuse FLAIR/T2W hyperintense signal involving both gray and white matter. The right cerebral hemisphere appeared normal. The left half of the brainstem and right cerebellum showed atrophy [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d.
|Figure 1: (a-d) T2W axial image of the brain shows marked left-sided cerebral atrophy with altered signal of the involved brain parenchyma (double arrow) with ex vacuo dilatation of the ventricle (arrow), along with ipsilateral brainstem (arrow) and contralateral cerebellar atrophy (arrow), (d) T1IR coronal image depicting similar findings|
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The present case is unique due to ipsilateral brainstem and contralateral cerebellar atrophy. The plausible explanation of this finding could be the long-standing disease. Due to long-standing unilateral cerebral atrophy, there is interruption of cerebro–ponto–cerebellar fibers, and so contralateral cerebellar diaschisis is noted. Ipsilateral brainstem atrophy can be attributed to Wallerian degeneration.
The prognosis of Rassmussen encephalitis is dismal. Treatment can only slow the progression of neurodegeneration.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
Rasmussen T, Olszewski J, Lloydsmith D. Focal seizures due to chronic localized encephalitis. Neurology 1958;8:435-45.
Tien RD, Ashdown BC. Crossed cerebellar diaschisis and crossed cerebellar atrophy: Correlation of MR findings, clinical symptoms, and supratentorial diseases in 26 patients Am J Roentgenol 1992;158:1155-9.