Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up (original) (raw)
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Rasmussen encephalitis: long-term outcome after surgery
Childs Nervous System, 2009
Background and purpose Rasmussen encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. RE causes are unknown, although evidence of an autoimmune process has been extensively described in the literature. Antiepileptic drugs are usually not effective to control seizures or cerebral atrophy; despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, for intractable seizures in RE patients with advanced disease, epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE. Materials and methods This work includes all the patients with RE evaluated from January 1995 to January 2008 by the Ribeirão Preto Epilepsy Surgery Program (CIREP), taking variables such as gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery, when done; duration of epilepsy; surgery complications; follow-up duration; anatomo-pathological findings; post-surgery seizure; language and cognitive outcome; and anti-epileptic drug treatment after surgery into account. Results Twenty-five patients were evaluated; thirteen were female. Mean age of epilepsy onset was 4.4 ± 2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p = 0.79), age at surgery (p = 0.24), duration of epilepsy (0.06), and follow-up (p = 0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p = 0.06). Immunomodulatory therapy was tried in 12 patients (48%). Twenty-three patients underwent surgery. The mean follow-up was 63.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (six patients), occasional hemigeneralized tonic–clonic seizures (three patients), and frequent tonic–clonic seizures (three patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. Eight patients presented post-operative cognitive decline, while only two patients had cognitive improvement. Comparing pre- and post-operative language deficits, 66.7% of the 12 patients with language disturbance did not improve after surgery. Conclusions This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Patients were divided into two groups: fast evolution and slow evolution to hemiparesis and epilepsia partialis continua. These groups may represent different RE substrates. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left-side involvement presented with some language disturbance, which did not improve after surgery in 66.6% of patients. Cognitive evaluation showed that the majority of the patients did not have any significant improvement, and 38.1% had cognitive deterioration after surgery.
Avaliação e reabilitação neuropsicológica após lesão encefálica adquirida
2017
Symptoms of interhemispheric disconnection after acquired brain injury are unusual in the current clinical practice. Symptoms usually diminish spontaneously and may include tactile anomia, agraphia and left signs of competition between the hemispheres, such as "alien hand sign". This work presents a case report of a 33 year old patient who developed symptoms of interhemispheric disconnection and executive function defi cits after surgery for removal of a tumor in the pituitary region, as well as the evaluation of the applicability of the neuropsychological rehabilitation program for the cognitive complaints presented. The patient was evaluated with standardized neuropsychological instruments before starting rehabilitation and again close to the discharge from treatment. The case study was conducted by analyzing intervention data and the clinical outcome of the patient in the institutional records (results of neuropsychological evaluation examination and records of interventions in rehabilitation sessions). Throughout his evolution, the patient showed improvement in cognitive symptoms and demonstrated benefi ts from the training and strategies used for his adaptation and return to work and other previous activities. The study highlights the importance of conducting more studies of neuropsychological interventions for individuals with cognitive defi cits.
Nationwide survey (incidence, clinical course, prognosis) of Rasmussen’s encephalitis
Brain and Development, 2010
Purpose: Rasmussen's encephalitis (RE) is a progressive and catastrophic epileptic disorder caused by chronic localized encephalitis. We performed a nationwide survey of RE to assess the clinical picture, treatment effect, and prognosis of Japanese RE patients. Subjects & methods: The subjects were 27 patients (male:12; female:15) from 13 medical facilities. All of them satisfied the clinical and neuroimaging criteria for RE, including 14 pathologically proven cases. Results: They were divided into the childhood-onset rapidly progressive type (CORP, n = 19), and late-onset slowly progressive type (LOSP, n = 8). The mean age at epilepsy onset was 4 years and 4 months in CORP, and 16 years in LOSP. The mean period between the onset age of epilepsy and development of frequent seizures was 1 year and 4 months in the former, and 3 years and 4 months in the latter. The immunomodulatory treatment including high-dose steroid (n = 14) and high-dose intravenous immunoglobulin therapies (IVIgG, n = 12) achieved more than a 50% reduction in the seizure frequency in 5 (36%) and 4 (33%) patients, respectively. Eight and seven patients underwent focal cortical resection and functional hemispherectomy, leading to significant improvement in 5 of the 8 patients and excellent seizure control in all 7 patients, respectively. Conclusion: Although the high-dose steroid and IVIG therapies may have alleviated the exacerbation of seizures in those with RE, they could not halt the disease progression. Functional hemispherectomy is still the only curative therapy for RE, despite the fact that the early introduction of this procedure remains controversial.
MRI findings in the diagnosis and monitoring of rasmussen's encephalitis
Arquivos de Neuro-Psiquiatria, 2009
Rasmussen's encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. Magnetic resonance (MR) findings, associated with clinical data and electroencephalogram (EEG), may indicate the diagnosis and could be an indicative of prognosis. We studied 5 patients with Rasmussen's encephalitis, assessing clinical history and MR images. All patients had refractory focal seizures with a predominant motor component associated with hemispheric atrophy, that was proportional to severity of disease and neurological deficits in these patients. Gray and white matter abnormal signal on T2 MR images were found in patients who had hemiparesis. It was not related to the duration of the disease but to aggressiveness. MR proton spectroscopy in severe disease showed lactate and choline increase and decreased NAA, reflecting neuronal and axonal loss, gliosis and elevated membrane turnover and recent -crisis (not controlled). MR studies, in addition to help in diagnosis, may be useful for monitoring metabolic changes and progression of disease in Rasmussen's encephalitis.
Seizure, 2009
To describe the attributes of patients with Rasmussen's encephalitis (RE) seen in a tertiary epilepsy referral center in southern India and to enquire factors helpful in predicting responsiveness to immunotherapy. Methods: We diagnosed RE based on the European consensus criteria. To assess the factors that could potentially predict the natural course and therapeutic outcome, we subcategorized our patients according to age at onset (</> 6 years), duration from onset to presentation (</> 2 years), immunotherapy versus surgery, and early (2 years from the onset) versus late surgery. Results: The median age at disease onset of 19 patients was 6.0 years (range 2.3-13 years). Epilepsia partialis continua (EPC) and hemiparesis were noted in 14 (73.6%) and 16 (84.2%) patients, respectively. One patient, who presented with dysarthria due to tongue EPC, did not have hemiparesis despite having had the disease for over 15 years. The MRI findings in majority conformed to stage 3 of Bien classification. While 9/10 patients treated by surgery achieved seizure-freedom, only 1/11 patients who received immunotherapy did so. One patient expired due to subsequent development of contralateral hemispheric disease following successful hemispherectomy. None of the factors such as age at onset, age at presentation, presence/absence of antecedents, seizure burden, MRI stage predicted responsiveness to immunotherapy. Conclusion: This study from a developing country, in addition to substantiating the well known characteristics of RE, noted the following unusual findings: isolated lingual EPC abolished by focal cortical resection, bilateral RE, putaminal atrophy and absence of hemiparesis despite long standing disease.
Functional Hemispherotomy in a Patient with Rasmussen’s Encephalitis: A Case Report
Bangladesh Medical Research Council Bulletin
Background: Rasmussen’s encephalitis is a progressive disease with recurrent drug resistant epilepsy, unihemispheric brain atrophy, hemiplegia and cognitive decline. Objectives: This is a disconnection surgery which disconnects the diseased lobe from the normal lobe. This helps in development of the normal cerebral hemisphere and also the child becomes seizure free. Methods: A five year old boy was diagnosed with Rasmussen’s encephalitis. He underwent left sided transsylvian functional hemisherectomy. He had uneventful recovery. Results: Before surgery he had recurrent seizures, frequent drop attacks and right sided hemiplegia and impaired cognition. He used to take three drugs for the control of the seizure. After surgery his mental state had improved, seizures were controlled with only sodium valproate. Patient’s mother was happy that he had no drop attacks and was able to sit and speak. Conclusion: Functional hemispherectomy is a good option for drug resistant seizure in Ramussen...
Putaminal involvement in Rasmussen encephalitis
Pediatric radiology, 2006
Background: Rasmussen encephalitis (RE) is a rare devastating disease of childhood causing progressive neurological deficits and intractable seizures, typically affecting one hemisphere. Characteristic MRI features include progressive unihemispheric focal cortical atrophy and grey-or white-matter high-signal changes and basal ganglion involvement, particularly of the caudate nucleus. Objective: To analyse the pattern of involvement of different brain structures in a series of patients with RE and to attempt clinical correlation. Materials and methods: We reviewed the medical records and neuroimaging data of 12 patients diagnosed with RE satisfying the European Consensus Statement diagnostic criteria. Results: The disease manifested as seizures in all patients and was refractory; epilepsia partialis continua was a notable feature (nine patients). Hemiparesis of varying grades was noted in all but one patient; none had extrapyramidal signs. Neuroimaging showed cortical involvement in the insular/periinsular regions in 11 patients. Caudate atrophy was noted in ten patients. Putaminal atrophy was seen in nine patients, six of whom had additional hyperintense signal changes. Conclusions: Our study highlights frequent putaminal atrophy and signal changes in RE, which suggests a more extensive basal ganglion involvement than emphasized previously. Recognition of putaminal changes may be a useful additional tool in the radiological diagnosis of RE.
Neuroplasticity in play: Outcomes after Hemispherectomy in Rasmussen Encephalitis
Introduction: A key property of the brain is its capacity to change after injury to enable at least some behavioral compensation. There are spontaneous reparative changes following injury, some of these changes are sufficient to support significant functional recovery. Rasmussen Encephalitis: Rasmussen Encephalitis is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Hemispherectomy in one of its modern variants offers a very high chance of seizure freedom and it is highly probable that the unaffected hemisphere compensates and takes over the functions of the removed hemisphere. Outcomes after Hemispherectomy: We review the some cases of positive outcomes after removal of a hemisphere in patients suffering from Rasmussen encephalitis. Discussion: Neuroplasticity is the key for a functional motor shift to the normal hemisphere to happen. Removal of a hemisphere dramatically illustrates the resilience of the brain to extensive damage. Conclusion: Research on the basic principles of brain plasticity is leading to new approaches to treating the injured brain. The power of neuroplasticity that is play in RE can be harnessed for intervention and rehabilitation in the field of neurology.
Perfil clinico de pacientes internados em unidades de neurologia
Revista Enfermagem Atual In Derme, 2019
Objetiva-se identificar o perfil clínico e sociodemográfico de pacientes internados em unidade de neurologia. Trata-sede um Estudo documental, retrospectivo, com abordagem quantitativa. A pesquisa foi realizada em um Hospital Terciário,localizado em Fortaleza, entre o período de dezembro de 2017 e março de 2018, tendo uma amostra de 184 prontuários.Os dados foram coletados por meio um instrumento com perfil sociodemográfico e clínico e analisados por meio de umSoftware estatístico. Houve predominância do sexo masculino representando 83,2%, com idade média entre 38,3, de corparda (77,7%) e natural do interior (60,3%) do Ceará. Os diagnósticos médicos mais encontrados foram o de traumatismocranioencefálico (54,8%) e trauma raquimedular (26%). Quanto ao desfecho dos pacientes, destaca-se que 88% obtiveramalta hospitalar, sendo que 28,8% apresentaram complicações neurológicas. Com uma média de 26 dias de internação hospitalar. Conclui-se o destaque da importância das tecnologias para ad...