Multiphasic presentation of Rasmussen's encephalitis

Rasmussen's encephalitis is a rare, chronic inflammatory disorder of unknown cause, characterised by drug‐resistant focal epilepsy that may rarely present in adolescence or adulthood. We present a case of Rasmussen's encephalitis with prominent recurrent fluctuation in symptoms and well‐documented fluctuating changes on MRI, adding to the spectrum of diversity of Rasmussen's encephalitis.     

Rasmussen's encephalitis: a role for autoimmune cytotoxic T lymphocytes

The present review describes advances in Rasmussen's encephalitis (also known as Rasmussen's syndrome), an unihemispheric intractable epileptic disease with persistent inflammation. Specific attention is given to the recent recognition of cytotoxicity by CD8+/granzyme-B-positive T lymphocytes as a new pathogenic mechanism of neuronal damage.     

Rasmussen脑炎一例临床病理分析

目的探讨Rasmussen脑炎的发病机制、临床特征、影像学特点、病理学特点及治疗方案。方法对1例Rasmussen脑炎的临床表现、影像学、组织形态学和免疫表型进行分析。结果临床、MRI图像及病理组织学特征均符合Rasmussen脑炎的诊断。结论 Rasmussen脑炎是一种罕见的可引起难治性癫疒间、偏瘫及神经功能损害的慢性进行性炎症,尽早行大脑半球切除术是目前治疗该病的最有效方法。     

Positive response to immunomodulatory therapy in an adult patient with Rasmussen's encephalitis

Rasmussen's encephalitis (RE) is a rare and progressive neurologic condition of uncertain etiology that typically has a childhood onset. The authors describe a 45-year-old woman with adult-onset progressive aphasia, right hemiparesis, severe drug refractory epilepsy, and left cerebral hemisphere atrophy. High-dose corticosteroids and plasmapheresis were not effective. She improved with high-dose therapy with human IV immunoglobulin.     

Extrarolandic electroclinical findings in the evolution of adult-onset Rasmussen's encephalitis

Rasmussen's encephalitis (RE) is a rare immunomediated disorder characterized by unilateral hemispheric atrophy, drug-resistant focal epilepsy, and progressive neurological deficits. Its onset typically occurs in childhood, though it has also been reported in adult age (A-RE) with atypical clinical features. The aim of this study was to describe the electroclinical features in a group of seven patients with A-RE.We retrospectively studied seven women aged 23–43 years (mean: 32.1 years) with a diagnosis of RE according to commonly accepted diagnostic criteria. All the patients were clinically evaluated and underwent prolonged video-EEG monitoring, laboratory investigations, and high-resolution MRI follow-up.All the patients displayed an ictal electroclinical pattern whose evolution varied. We identified an early phase characterized by polymorphic ictal electroclinical manifestations (temporal semiology in five cases, frontal in one, and parietal in the remaining case) and a late phase clinically characterized by viscerosensitive phenomena followed by somatosensitive signs, experiential symptoms, and motor signs in all the cases. In the late phase, the ictal EEG pattern was characterized by monomorphic, pseudorhythmic, repetitive slow-wave theta activity over the frontal and central regions, with ipsilateral propagation and/or secondary spreading to contralateral perisylvian structures. Patients were treated with a combination of AEDs and immunotherapy (steroids and IVIg); epilepsy surgery was performed in 3 cases.Our results show that A-RE is characterized by early and late clinical- and EEG-different features which may reflect a progressive involvement of a specific “extrarolandic” network in the advanced phase of the disease and may suggest that the electroclinical expression of RE varies according to the different stages of the pathological process.     

Utility of Ictal SPECT in the presurgical evaluation of Rasmussen's encephalitis

BACKGROUND: Rasmussen's encephalitis is a progressive disease characterized by severe progressive neurological and cognitive deficits. It is a difficult condition to treat with conventional medical treatment and requires, in most of the cases, surgical intervention. METHODS: We present the case of a patient with Rasmussen's encephalitis who presented with epilepsia partialis continua, who did not respond to antiepileptic treatment and was evaluated for possible surgical intervention. RESULTS: The electroencephalographic studies revealed bihemispheric involvement during seizures, although magnetic resonance imaging (MRI) showed a marked right hemispheric atrophy. Hyperperfusion over the right hemisphere was seen with the performance of ictal single photon emission computed tomography (SPECT), which finally guided a right functional hemispherectomy and subsequent seizure control. CONCLUSION: Ictal SPECT could be a useful tool in the armamentarium of tools used for the localization of the epileptogenic focus in patients with Rasmussen's encephalitis and other localization-related epilepsies.     

Finding on brain MRI mimicking focal cortical dysplasia in early Rasmussen's encephalitis: a case report and review

Introduction  

Rasmussen's encephalitis (RE) is one of the important causes of refractory seizure. The most impressive clinical manifestation of RE is epilepsia partialis continua (EPC). Others include progressive hemiparesis and neuropsychological deterioration. Currently, the best approach to RE is hemispherectomy.     

Neuroimaging follow-up in a case of Rasmussen's encephalitis with dyskinesias.

We describe a case of adult-onset biphasic Rasmussen's encephalitis who presented seizures and left dyskinesias at the onset and, after 1 year, language disorder. Serial MRI and [(18)F] FDG-PET scans were performed showing involvement of the right cerebral hemisphere in the first phase and of the contralateral one in the second.     

Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology.

OBJECTIVE: To correlate MRI and histopathologic findings in patients with Rasmussen's encephalitis (RE). PATIENTS AND METHODS: MRI features of 10 patients with RE were studied on serial scans. In surgical specimens from these patients, densities of T lymphocytes, microglial cells and nodules, and reactive astrocytes were evaluated. RESULTS: Densities of T cells, microglial nodules, and astrocytes were inversely correlated to disease duration. MRI abnormalities had a focal onset and spread across one hemisphere. The following course of MRI abnormalities in a given brain region was observed: on very early scans, the cortex was swollen and showed a hyperintense T2/fluid-attenuated inversion recovery signal. Consecutively, progressive atrophy of the affected hemisphere occurred. Correlation of MRI features with quantitative histopathology revealed that there was a higher number of T cells and reactive astrocytes in the earlier MRI stages compared with the late (merely atrophic) stage. CONCLUSION: These data suggest a disease course in RE with the highest inflammatory intensity in the early stages and a subsequent decrease in inflammation. The MRI abnormalities and their characteristic sequence may help to identify patients with RE and to obtain informative biopsies.     

GluR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis.

Eight patients with Rasmussen's encephalitis, 40 patients with noninflammatory focal epilepsy, 104 patients with various neurologic diseases, and 16 healthy donors were tested for the prevalence of antibodies against the GluR3 receptor in serum and CSF. Reactivities against different peptides derived from various portions of this glutamate receptor subtype were detectable in a significantly higher number of patients with focal epilepsy than in those with other neurologic diseases, but they were not specific for the diagnosis of Rasmussen's encephalitis.     

Amygdala atrophy is prominent in early Alzheimer's disease and relates to symptom severity

Despite numerous studies on the role of medial temporal lobe structures in Alzheimer's disease (AD), the magnitude and clinical significance of amygdala atrophy have been relatively sparsely investigated. In this study, we used magnetic resonance imaging (MRI) to compare the level of amygdala atrophy to that of the hippocampus in very mild and mild AD subjects in two large samples (Sample 1 n=90; Sample 2 n=174). Using a series of linear regression analyses, we investigated whether amygdala atrophy is related to global cognitive functioning (Clinical Dementia Rating Sum of Boxes: CDR-SB; Mini Mental State Examination: MMSE) and neuropsychiatric status. Results indicated that amygdala atrophy was comparable to hippocampal atrophy in both samples. MMSE and CDR-SB were strongly related to amygdala atrophy, with amygdala atrophy predicting MMSE scores as well as hippocampal atrophy, but predicting CDR-SB scores less robustly. Amygdala atrophy was related to aberrant motor behavior, with potential relationships to anxiety and irritability. These results suggest that the magnitude of amygdala atrophy is comparable to that of the hippocampus in the earliest clinical stages of AD, and is related to global illness severity. There also appear to be specific relationships between the level of amygdala atrophy and neuropsychiatric symptoms that deserve further investigation.