Chronic focal encephalitis, or rasmussens encephalitis, is a rare and devastating inflammatory brain disease that can lead to the destruction or. Tobias loddenkemper, md director, clinical epilepsy research. Rasmussen encephalitis radiology reference article. Rasmussens ence phalitis re is a rare, progressive, chronic encephalitis characterised by drugresistant epilepsy, progressive hemiparesis and mental impairment. Rasmussens encephalitis re is a rare pediatric neurological disorder, the etiology of which remains unclear. Distinguishing between these etiologies is critical to guide appropriate surgical intervention, but in this case the lack of obvious hemiatrophy complicated accurate diagnosis. Here, we report a case of rasmussen encephalitis with an update on recent diagnostic criteria and emphasis on differential diagnoses which can be excluded on imaging.
Diagnosis and staging of rasmussens encephalitis by. Magnetic resonance imaging mri shows changes of cerebral atrophy in the avected hemisphere. Hemispherectomy remains the only treatment that leads to seizure freedom for this refractory epileptic syndrome. In this study, we analyzed the expression of epsteinbarr virus ebv. Rasmussen encephalitis was first described by american neurologist theodore brown rasmussen 19102002 in 1958 2. Cytotoxic t lymphocytes are a major driver of pathogenicity in rasmussen encephalitis. Examples of magnetic resonance imaging mri scans from patients with rasmussen encephalitis re or cortical dysplasia cd. Simply select your manager software from the list below and click on download. Rasmussen encephalitis nord national organization for rare. Rasmussen encephalitis is a rare, progressive disease beginning in the first decade in previously normal children rasmussen et al. As the disease is resistant to drug treatments, it frequently requires surgical. Rasmussen encephalitis is a rare neurological disease accompanied by inflammation and t cell infiltration in the brain. Rasmussens encephalitis, mri, spectroscopy, epilepsy.
Longitudinal study of mrs metabolites in rasmussen encephalitis. It has been speculated that the immunopathogenesis of re involves damage to neurons, which eventually leads to the occurrence of re. Rasmussen syndrome, also known as rasmussen encephalitis, is typically associated with volume loss of the affected hemisphere of the brain. However, detection in adults is increasing with routine mri investigations for intractable seizures 4. The condition has previously been studied by various imaging modalities including mr imaging and mr spectroscopy.
English and colleagues describe five children with rasmussens encephalitis in whom spect imaging demonstrated an area of hypoperfusion and hypometabolism corresponding to the anatomical localization of the. To compare the rate of abnormal brain metabolism by fdgpetct to other paraclinical findings and to describe brain metabolism patterns in autoimmune encephalitis ae. It is characterized by the sudden appearance of refractory partial seizures, often with epilepsia partialis continua, or less commonly as complex partial status. Anatomic changes of atrophy may be subtle in earlier phases of the disease, and progressive changes on serial scans may be difficult to detect. Longitudinal study of mrs metabolites in rasmussen. Rasmussens encephalitis re is an inflammatory encephalopathy of unknown cause defined by seizures with progressive neurological disabilities. Magnetic source imaging, like mrfdgpet fusion imaging, allows the detection of many subtle abnormalities that may otherwise go. We present neuroradiological findings in children with re.
The illness affects a single cerebral hemisphere and generally occurs in children under the age of 15. Rasmussens encephalitis re is a chronic inflammatory brain disorder that causes frequent seizures and unilateral hemispheric atrophy with progressive neurological deficits. Despite major advances in the diagnosis of infectious diseases 3 and increasing recognition of. Rethinking the magnetic resonance imaging findings in early.
Meg magnetoencephalography provides a noninvasive tool to study epilepsy and brain function. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. A pretreatment, semiquantitative interictal brain perfusion single photon emission computed tomography spect scan using 99tcm hmpao hexamethylene propylene amine oxime showed hypoperfusion in the clinically. Patterns of cerebral glucose metabolism in early and late. Patient with rightsided temporal lobe epilepsy and mesial temporal sclerosis on magnetic resonance imaging right shows increased uptake of 11 cpbr28 left ipsilateral to seizure focus. Rasmussens encephalitis re is an acquired progressive unihemispheric. Automated mri volumetric analysis in patients with. It occurs mainly in children most cases are seen in six to sevenyearsold children. We present a child with rasmussen encephalitis and highlight the pitfalls of diagnosis when magnetic resonance imaging mri is negative for atrophy. Encephalitis of unclear origin diagnosed by brain biopsy. Rasmussen encephalitis causes, symptoms, diagnosis.
If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. Encephalitis is a neurologic syndrome characterized by variable neurologic dysfunction that is accompanied by inflammation of the brain parenchyma. Initial diagnosis and treatment are based on the clinical presentation as well as antibody profiles and mri. Diagnosis and staging of rasmussens encephalitis by serial mri and histopathology. Mri features of 10 patients with re were studied on serial scans. Freesurfer is useful for early detection of rasmussens. Rasmussen syndrome an overview sciencedirect topics. Rasmussen encephalitis re, also called rasmussen syndrome, is a rare, progressive, chronic encephalitis inflammation of the brain affecting one hemisphere one side of the brain. Freesurfer, an open source software suite for processing and analyzing human mri, was used to measure cortical.
Mri findings in the diagnosis and monitoring of rasmussens encephalitis. Jci humanized mouse model of rasmussens encephalitis. Serial perfusion brain tomographic scans detect reversible. Most cases 85% cases occur in children under the age of 10 years 1. The threshold function of another image processing software scion image for. Rethinking the magnetic resonance imaging findings in. Rasmussen encephalitis is a progressive inflammatory process with difficulttocontrol focal or lateralized seizure activity, leading to hemispheric dysfunction and atrophy in advanced stages.
The spectra were postprocessed using software supplied by the machine manufacturer. Rethinking the magnetic resonance imaging findings in early rasmussen encephalitis. However, around 10% of all cases are adolescents and adults. Rasmussen encephalitis, also known as chronic focal encephalitis, is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. Rasmussen encephalitis is a chronic, progressive encephalitis that manifests as an abruptonset, intractable seizure disorder in.
Rasmussens encephalitis is a rare autoimmune disease that primarily affects children and can lead to seizures. Neuroimaging shows the progressive damage of the affected hemisphere. Mri was performed in all patients, fluorodeoxyglucose positronemission tomography pet in. Patients with re at the university of alberta, edmonton, ab, canada, were identified and analyzed by neuroimaging, neuropsychological.
Rasmussen encephalitis, sometimes referred to as rasmussen syndrome, is a. We infer that crossed cerebellar atrophy is a distinctive feature of longterm rasmussen encephalitis evolution. Introduction limbic encephalitis le is an immunerelated, sometimes paraneoplastic process of the central nervous system. Imaging plays an important role in diagnosis by demonstrating focal or unihemispheric involvement and excluding other possible causes. Surgical removal or disconnection of the affected cerebral hemisphere is currently the only intervention that effectively stops the seizures. Herein, the pathogenesis of re was investigated focusing on inflammasome activation in the brain. Viral infection may be a critical factor in triggering re immunopathogenesis.
Pdf mri findings in the diagnosis and monitoring of rasmussens. T lymphocytes are a major driver of pathogenicity in rasmussen encephalitis. As the disease is resistant to drug treatments, it frequently. Multimodality software, nuclear diagnostics, uk to compare the scans. In surgical specimens from these patients, densities of t lymphocytes, microglial cells and nodules, and reactive astrocytes were evaluated. Rasmussens encephalitis re is a medically refractory epilepsy that usually begins. Here the authors show that the severity of this disease correlates with. Abnormal brain metabolism on fdgpetct is a common early. Role of 18ffdgpet imaging in the diagnosis of autoimmune. Request pdf diagnostic imaging in cases of rasmussens encephalitis. Automated 3d mri volumetry reveals regional atrophy. Although the exact cause of this disorder is not known, viral or postviral etiology have been postulated.
Diagnostic imaging in cases of rasmussens encephalitis. Scans were semiquantitatively assessed for t2 a and c and flair b and d signal changes. Tractography of corticopontocerebellar cpc pathways 2 figure 2, c and d demonstrated marked involvement of the right tract, suggesting that a dyingback mechanism of efferent cpc fibers may cause crossed cerebellar atrophy. Rasmussen encephalitis can be diagnosed if either all three criteria of part a or two of three criteria of part b are present. Elevated expression of ebv and tlrs in the brain is. Rasmussens encephalitis is a chronic inflammation of the brain that leads to progressive neurologic deficits.
Abstract rasmussens encephalitis is a devastating syndrome of multifocal brain dysfunction and focal. The clinical and imaging findings are consistent with the late stage of rasmussen encephalitis, which is a rare disorder of the central nervous system characterized by chronic encephalitis of one hemisphere of the brain. Rasmussens ence phalitis, is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motor skills and speech, hemiparesis weakness on one side of the body, encephalitis inflammation of the brain, and dementia. Magnetic resonance mr findings, associated with clinical data and electroencephalogram eeg, may indicate the diagnosis and could be an indicative of prognosis. Serial magnetic resonance imaging findings of rasmussens encephalitiscase report. Until now, quantitative parameters describing the course of the disease on mri require manual intervention and are, therefore, time. Next article role of 18ffdgpet imaging in the diagnosis of autoimmune encephalitis. When it is combined with structural imaging, it is known as magnetic source imaging msi. Rasmussens encephalitis an overview sciencedirect topics.
Clinical application and evaluation of the bien diagnostic criteria for rasmussen encephalitis. This study investigated the diagnostic value of integrated 18ffdgpetmri in the diagnostic workup of patients with le for a cerebral and wholebody imaging. Volumetric analysis could also be included as part of followup for patients with rasmussen encephalitis to assess disease progression. The re case a and b was a year old with a fiveyear history of seizures originating from the right hemisphere. Rasmussens encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures.
Our aim was to apply automated quantitative volumetric mr imaging analyses to patients diagnosed with rasmussen encephalitis, to determine the predictive value of lobar volumetric measures and to assess. We present a child with rasmussen encephalitis and. In parallel, the imaging community needs to refine standard procedures for acquisition and reading of pet imaging data in autoimmune encephalitis, and. Mrfdgpet fusion imaging is performed with software that registers mr images with fdg pet images.
Most rasmussen encephalitis 85% cases occur in children under the age of 10 years 3. Professor and neurosurgical director, pediatric epilepsy surgery program. A 39yearold man with advanced adultonset rasmussens encephalitis was treated with prednisolone and longterm, highdose, human intravenous immunoglobulin. Temporal lobe epilepsy and pet imaging of translocator protein. Voxelbased comparison of the patient data with an agematched control group using spm software. To correlate mri and histopathologic findings in patients with rasmussen s encephalitis re. It typically involves only one cerebral hemisphere, which becomes atrophic. Patient with temporal lobe epilepsy with higher 11 cpbr28 uptake ipsilateral than contralateral to seizure focus. It is not to be confused with a rasmussen aneurysm. Evidence for resident memory t cells in rasmussen encephalitis. Pdf rasmussens encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. Mri findings in the diagnosis and monitoring of rasmussen.
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