|Year : 2020 | Volume
| Issue : 3 | Page : 205-207
A case of Rasmussen's encephalitis with an atypical presentation
MA Rahman, NS Vithal Rao, Lakshmi Manasa Perumalla
Department of Paediatrics, Siddhartha Medical College, Vijayawada, Andhra Pradesh, India
|Date of Submission||17-Jun-2020|
|Date of Acceptance||28-Jun-2020|
|Date of Web Publication||30-Sep-2020|
Dr. Lakshmi Manasa Perumalla
Department of Paediatrics, Siddhartha Medical College, Vijayawada, Andhra Pradesh - 520 013
Source of Support: None, Conflict of Interest: None
Rasmussen's encephalitis (RE) is a chronic neurological disorder characterized by unilateral inflammation of the cerebral cortex leading to severe focal seizures, progressive loss of cognition, and neurological deficits in the affected hemisphere. We present a rare case of RE in a child with global developmental delay and cerebral atrophy. Neuroimaging helped in clinching the diagnosis of the disease. Immunomodulators may slow the progression of the disease; hence, it needs to be identified early and treated promptly. Functional cerebral hemispherectomy is the only treatment for drug-resistant seizures, but it has its functional compromise.
Keywords: Focal seizures, hemispherectomy, immunomodulators, Rasmussen's encephalitis
|How to cite this article:|
Rahman M A, Vithal Rao N S, Perumalla LM. A case of Rasmussen's encephalitis with an atypical presentation. J NTR Univ Health Sci 2020;9:205-7
|How to cite this URL:|
Rahman M A, Vithal Rao N S, Perumalla LM. A case of Rasmussen's encephalitis with an atypical presentation. J NTR Univ Health Sci [serial online] 2020 [cited 2022 Aug 11];9:205-7. Available from: https://www.jdrntruhs.org/text.asp?2020/9/3/205/296832
| Introduction|| |
Rasmussen's encephalitis (RE) was first described by Theodore Rasmussen in 1958 as a chronic inflammatory disease, usually affecting one brain hemisphere. It is usually reported in children aged <10 years, though it can be seen in adolescents and adults as well. The early institution of immunotherapy helps in improving the outcome and altering the natural course of the disease. Here we report a case of a young boy with RE based on imaging features.
| Case Report|| |
A 3-year-old boy born of 3rd-degree consanguineous marriage presented with complaints of multiple episodes of tonic-clonic seizures of the left upper and lower limb since the last 2 weeks, each episode was lasting for 5–15 min with a prolonged postictal state. The child is a known case of global developmental delay with a seizure disorder. He had no history of blood transfusions, neurosurgery, traumatic head injury, proven allergies, and family history of epilepsy. On examination, the child was disoriented. His vitals were stable. Central nervous system examination showed left-sided hemiparesis with exaggerated tendon reflexes on the left side. There were no signs of meningeal irritation, and cranial nerves were intact. Cerebrospinal fluid analysis was normal, MRI showed diffuse cerebral atrophy with gyral swelling involving the entire right cerebral hemisphere; the adjacent sulcal spaces, and the Sylvian fissure appeared compromised due to gyral swelling. There was a restriction of diffusion in the corresponding DW images. On T2 head of the caudate and lentiform nucleus appeared bulky on the right side compared to the left side resembling MRI stage 1 of RE [Figure 1]. Considering all the above features, a diagnosis of an acute phase of RE was made, and the child was started on intravenous steroids. The seizure frequency was controlled over 3 weeks.
|Figure 1: MRI (diffusion-weighted) showing diffuse cerebral atrophy with gyral swelling involving the entire right cerebral hemisphere; the adjacent sulcal spaces, and the Sylvian fissure appears compromised due to gyral swelling|
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| Discussion|| |
RE is a rare chronic inflammatory progressive neurological disorder. The median age of onset is 6 years. They usually present with focal motor seizures followed at varying time intervals by progressive loss of ipsilateral motor functions and cognitive impairment. Etiologically, there are different theories for the pathogenesis-autoimmune against type-3 glutamate receptors or infective. The diagnosis of RE is based on clinical, radiological, and pathological features with emphasis laid on clinical and radiological features [Table 1]. Bien et al. proposed a three-stage natural history of RE based on clinical findings and four-stage course of disease based on MRI findings [Table 2].
Three distinct features of epilepsy noted in RE are a) polymorphism of seizures in a given patient, b) frequent occurrence of epilepsia partialis continua (EPC), and c) medical intractability of seizures. Imaging plays a vital role in diagnosis by demonstrating unihemispheric involvement (focal cortical atrophy) and excluding other possible causes [see [Table 1]. It is essential to differentiate RE from other unihemispheric epileptic syndromes (hemimegalencephaly, tuberous sclerosis, Sturge– Weber syndrome More Details), EPC due to metabolic disorders (diabetes, renal, or hepatic encephalopathy), progressive neurological diseases (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes [MELAS], Alpers syndrome), inflammatory/infectious diseases (multiple sclerosis, subacute sclerosing panencephalitis [SSPE], cerebral vasculitis in systemic lupus erythematosus (SLE)].
Management of RE is challenging and disappointing. Steroids, immunomodulators, plasma exchange, and immunoglobulins have been used in the acute phase of the disease with limited success. Surgical treatment in the form of functional hemispherectomy is needed in resistant cases. A modified Transylvanian keyhole functional hemispherectomy has evolved, which aims to interrupt the connection of cortex with basal ganglia and contralateral hemisphere. Our patient was started on intravenous steroids and antiepileptics, which resulted in reduced episodes of seizures.
In our case, a 3-year-old boy with diffuse cortical atrophy secondary to hypoxic-ischemic encephalopathy presented with unilateral focal seizures 3 years after birth, with clinical and radiological features suggestive of an acute phase of RE. Such a case has not been reported so far. It is essential to identify such a case and differentiate it from bilateral RE where unaffected hemispheres undergo progressive and slow atrophy in later years.
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, Lloyd-Smith D. Focal seizures due to chronic localized encephalitis. Neurology 1958;8:435-45.
McLachlan RS, Girvin JP, Blume WT, Reichman H. Rasmussen′s chronic encephalitis in adults. Arch Neurol 1993;50:269-74.
Rogers SW, Andrews PI, Gahring LC, Whisenand T, Cauley K, Crain B, et al
. Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. Science 1994;265:648-51.
Power C, Poland SD, Blume WT, Girvin JP, Rice GP. Cytomegalovirus and Rasmussen's encephalitis. Lancet 1990;336:1282-4.
Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, et al
. Rasmussen's encephalitis: Clinical features, pathobiology, and treatment advances. Lancet Neurol 2014;13:195-205.
Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, et al
. The natural history of Rasmussen's encephalitis. Brain 2002;125(Pt 8):1751-9.
Bien CG, Urbach H, Deckert M, Schramm J, Wiestler OD, Lassmann H, et al
. Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology. Neurology 2002;58:250-7.
Hart YM, Cortez M, Andermann F, Hwang P, Fish DR, Dulac O, et al.
Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): Effect of high-dose steroids or immunoglobulins in 19 patients. Neurology 1994;44:1030-6.
Schramm J, Kral T, Clusmann H. Transsylvian keyhole functional hemispherectomy. Neurosurgery 2001;49:891-900.
Larionov S, König R, Urbach H, Sassen R, Elger CE, Bien CG. MRI brain volumetry in Rasmussen encephalitis: The fate of affected and “unaffected” hemispheres. Neurology 2005;64:885-7.
[Table 1], [Table 2]