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Quantitative Imaging in Medicine and... May 2020
PubMed: 32489942
DOI: 10.21037/qims.2020.03.17 -
Cureus Oct 2023Usually affecting one hemisphere of the brain, Rasmussen's encephalitis (RE) is a persistent inflammatory disease of unclear origin. Rasmussen and colleagues presumed a... (Review)
Review
Usually affecting one hemisphere of the brain, Rasmussen's encephalitis (RE) is a persistent inflammatory disease of unclear origin. Rasmussen and colleagues presumed a viral etiology of the sickness in their first description. Later, the condition was linked to autoantibodies that were in the blood. Recently, it was shown that the cause of RE was a cytotoxic T-cell reaction to neurons. RE may be identified histopathologically by cortical inflammation, neuronal degeneration, and cerebral hemispheric-specific gliosis. The hemisphere is affected by increasing multilocular inflammation. To diagnose patients sooner and to evaluate whether the aforementioned phenomena are primary or secondary, it is essential to continue the search for a primary immunological or viral component. This information is crucial for determining the effectiveness of immunotherapy. RE-related seizures can only now be managed surgically. The only procedure that works is complete hemispheric disconnection (hemidisconnection), which may be done as either a (functional) hemispherectomy or hemispherectomy. Although thalidomide has been anecdotally reported, its safety profile prevents it from being used as a first-line treatment despite having a noticeable effect on the frequency and severity of seizures. Finding the disease's root causes more quickly by combining descriptive clinical studies, genetic testing, and early histological evaluation of RE tissue specimens to check for viral and autoimmune pathogenesis. Creating appropriate in vitro or animal models will enable the study of causality, perhaps directing clinical trials.
PubMed: 38022088
DOI: 10.7759/cureus.47698 -
Neurologia Medico-chirurgica Apr 2023Hemispherotomy is a radical treatment for drug-resistant epilepsy that targets developmental, acquired, and progressive diseases with widespread epileptogenic regions in...
Hemispherotomy is a radical treatment for drug-resistant epilepsy that targets developmental, acquired, and progressive diseases with widespread epileptogenic regions in one cerebral hemisphere. Currently, two main approaches are utilized after repeated improvements: lateral and vertical approaches. With the lateral approach, the surgical field is wide, and the approach to the lateral ventricle is relatively easy. On the other hand, the vertical approach has the advantage of reducing intraoperative bleeding and operating time as the resection line of the radial fiber is short, and understanding the three-dimensional anatomy is relatively easy. The lateral approach is generally used for atrophic lesions, whereas the vertical approach is for hypertrophic lesions. Hemispherotomy is expected to not only suppress epileptic seizures but also improve psychomotor development by protecting the unaffected cerebral hemisphere. However, this method is one of the most invasive surgeries in epilepsy surgery, and it is important to fully consider its indications. Furthermore, understanding the neural fiber pathway is important for actual surgery.
Topics: Humans; Hemispherectomy; Treatment Outcome; Epilepsy; Drug Resistant Epilepsy; Seizures
PubMed: 36682794
DOI: 10.2176/jns-nmc.2022-0279 -
Epilepsia Dec 2019To examine longitudinal seizure and functional outcomes after hemispherectomy in adults and adolescents.
OBJECTIVE
To examine longitudinal seizure and functional outcomes after hemispherectomy in adults and adolescents.
METHODS
We reviewed 47 consecutive patients older than 16 years who underwent hemispherectomy between 1996 and 2016 at our center. Clinical, electroencephalographic (EEG), imaging, neuropsychological, surgical, and functional status data were analyzed.
RESULTS
Thirty-six patients were 18 years or older at surgery; 11 were aged between 16 and 18 years. Brain injury leading to hemispheric epilepsy occurred before 10 years of age in 41 (87%) patients. At a mean follow-up of 5.3 postoperative years (median = 2.9 years), 36 (77%) had Engel class I outcome. Longitudinal outcome analysis showed 84% seizure freedom (Engel IA) at 6 months, 76% at 2 years, and 76% at 5 years and beyond, with stable longitudinal outcomes up to 12 years from surgery. Multivariate analysis demonstrated that acute postoperative seizures and contralateral interictal spikes at 6-month follow-up EEG were associated with seizure recurrence. Patients who could walk unaided preoperatively and had no cerebral peduncle atrophy on brain magnetic resonance imaging were more likely to experience worsening of motor function postoperatively. Otherwise, postoperative ambulatory status and hand function were unchanged. Of the 19 patients who completed neuropsychological testing, 17 demonstrated stable or improved postoperative outcomes.
SIGNIFICANCE
Hemispherectomy in adults is a safe and effective procedure, with seizure freedom rates and functional outcome similar to those observed in children.
Topics: Adolescent; Adult; Electroencephalography; Female; Follow-Up Studies; Hemispherectomy; Humans; Male; Middle Aged; Neuropsychological Tests; Recovery of Function; Seizures; Treatment Outcome; Young Adult
PubMed: 31677151
DOI: 10.1111/epi.16378 -
Journal of Epilepsy Research Jun 2021Refractory epilepsy is when seizures are unresponsive to two or more medications. Hemispherectomy, one of the treatment options, is the complete removal or functional...
BACKGROUND AND PURPOSE
Refractory epilepsy is when seizures are unresponsive to two or more medications. Hemispherectomy, one of the treatment options, is the complete removal or functional disconnection of a cerebral hemisphere. Hemiparesis, a symptom of epilepsy, is defined as weakness of one side of the body. Patients with refractory epilepsy, who experience extreme seizure frequency, are subjected to hemispherectomy. This study focuses on finding the correlation between hemispherectomy and hemiparesis, discovering a pattern in its severity levels before and after surgery.
METHODS
Data was collected from 59 epileptic patients suffering from refractory epilepsy, who underwent hemispherectomy, at the King Faisal Specialist Hospital and Research Centre, from 1998 to 2014. Each patient was monitored over a period of 1-year post-surgery. In this study, we wanted to further explore the correlation between hemispherectomy and hemiparesis.
RESULTS
Upon analyzing the sample size, the level of hemiparesis pre and post hemispherectomy remained the same for 32 patients, of which 13 patients had mild levels pre-surgery and 19 patients had moderate to severe levels pre-surgery. However, 20 of the patients who had moderate to severe levels of hemiparesis before the surgery had either no hemiparesis or a mild level after surgery, which signified an improvement in their severity level. On the other hand, seven of the patients went from having no hemiparesis before the surgery to having moderate or severe levels of hemiparesis post-surgery. Although the results were clinically significant, they were not statistically significant as the -value obtained was 0.31.
CONCLUSIONS
After analyzing the results, it can be concluded that hemispherectomy does decrease the severity of hemiparesis in an epileptic patient, thereby improving his/her quality of life drastically.
PubMed: 34395221
DOI: 10.14581/jer.21005 -
Neurosurgery Jul 2022Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures.
BACKGROUND
Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures.
OBJECTIVE
To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution.
METHODS
Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020. We collected seizure history, postoperative outcomes, and functional data. Imaging was independently reviewed in a blinded fashion by 2 neurosurgeons and a neuroradiologist.
RESULTS
We analyzed 30 patients with RE who underwent 35 hemispherectomies (5 reoperations). Using Kaplan-Meier analysis, seizure-freedom rate was 81.5%, 63.6%, and 55.6% at 1, 5, and 10 years after surgery, respectively. Patients with shorter duration of hemiparesis preoperatively were less likely to be seizure-free at follow-up (P = .011) and more likely to undergo reoperation (P = .004). Shorter duration of epilepsy (P = .026) and preoperative bilateral MRI abnormalities (P = .011) were associated with increased risk of reoperation. Complete disconnection of diseased hemisphere on postoperative MRI after the first operation improved seizure-freedom (P = .021) and resulted in fewer reoperations (P = .034), and reoperation resulted in seizure freedom in every case.
CONCLUSION
Obtaining complete disconnection is critical for favorable seizure outcomes from hemispherectomy, and neurosurgeons should have a low threshold to reoperate in patients with RE with recurrent seizures. Rapid progression of motor deficits and bilateral MRI abnormalities may indicate a subpopulation of patients with RE with increased risk of needing reoperation. Overall, we believe that hemispherectomy is a curative surgery for the majority of patients with RE, with excellent long-term seizure outcome.
Topics: Electroencephalography; Encephalitis; Hemispherectomy; Humans; Inflammation; Reoperation; Retrospective Studies; Seizures; Treatment Outcome
PubMed: 35544031
DOI: 10.1227/neu.0000000000001958 -
Translational Neuroscience 2020To investigate the effect and medical imaging of modified hemispherectomy on patients with infantile hemiparesis and medically refractory epilepsy.
OBJECTIVE
To investigate the effect and medical imaging of modified hemispherectomy on patients with infantile hemiparesis and medically refractory epilepsy.
PATIENTS AND METHODS
Forty-three patients with infantile hemiparesis and refractory epilepsy who underwent hemispherectomy were enrolled. The treatment effect and medical imaging were analyzed.
RESULTS
Anatomical hemispherectomy was successfully performed in all patients (100%). In all patients, the muscular tension decreased and the contracted limbs relaxed. In the pathological examination of the resected brain tissue, secondary cicatricial gyri with concomitant cortical dysplasia was present in 36 cases and polycerebellar gyrus malformation and porencephalia in the other 7 cases. Followed up for 7-15 years (mean 11.3), all patients were alive without a long-term sequela. Epilepsy was satisfactorily controlled, with complete seizure relief in 39 cases (91%) classified as Engel I and basic control in the other 4 (9%) defined as Engel II. The posthemispherectomy medical imaging demonstrated that the intracranial space on the operative side shrank, and the healthy cerebral hemisphere shifted markedly toward the hemispherectomy side, with expanded lateral ventricle on the healthy side and thickened skull and enlarged frontal sinus on the operative side. After 4-5 years, the intracranial space on the operative side disappeared in 75% of the patients, demonstrating enlarged cerebral peduncle on the healthy side.
CONCLUSION
Further modified hemispherectomy in patients with infantile hemiparesis and medically refractory epilepsy demonstrated markedly ameliorated effects on epilepsy control and the prevention of superficial cerebral hemosiderosis in the long-term follow-up.
PubMed: 33335778
DOI: 10.1515/tnsci-2020-0145 -
Brain & Spine 2023Hemispherectomy/hemispherotomy has been employed in the management of catastrophic epilepsy. However, initial reports on the associated mortality and morbidity raised... (Review)
Review
INTRODUCTION
Hemispherectomy/hemispherotomy has been employed in the management of catastrophic epilepsy. However, initial reports on the associated mortality and morbidity raised several concerns regarding the technique's safety. Their actual, current incidence needs to be systematically examined to redefine hemispherotomy's exact role.
RESEARCH QUESTION
Our current study examined their incidence and evaluated the association of the various hemispherotomy surgical techniques with the reported complications.
MATERIAL & METHODS
A PRISMA-compliant systematic review and meta-analysis was performed. We searched PubMed, Scopus, and Web of Science until December 2022. Fixed- and random-effects models were employed. Egger's regression test was used for estimating the publication bias, while subgroup analysis was utilized for defining the role of the different hemispherotomy techniques.
RESULTS
We retrieved a total of 37 studies. The overall procedure mortality was 5%, with a reported mortality of 7% for hemispherectomy and 3% for hemispherotomy. The reported mortality has decreased over the last 30 years from 32% to 2%. Among the observed post-operative complications aseptic meningitis and/or fever occurred in 33%. Hydrocephalus requiring a shunt insertion occurred in 16%. Hematoma evacuation was necessary in 8%, while subgaleal effusion in another 8%. Infections occurred in 11%. A novel post-operative cranial nerve deficit occurred in 11%, while blood transfusion was necessary in 28% of the cases.
DISCUSSION AND CONCLUSION
Our current analysis demonstrated that the evolution from hemispherectomy to hemispherotomy along with neuroanesthesia advances, had a tremendous impact on the associated mortality and morbidity. Hemispherotomy constitutes a safe surgical procedure in the management of catastrophic epilepsies.
PubMed: 38021002
DOI: 10.1016/j.bas.2023.101766 -
Therapeutic Advances in Chronic Disease 2020Rasmussen's encephalitis (RE) is rare neurological diseases characterized as epilepsia partialis continua, invariably hemiparesis, and cognitive impairment. This disease... (Review)
Review
Rasmussen's encephalitis (RE) is rare neurological diseases characterized as epilepsia partialis continua, invariably hemiparesis, and cognitive impairment. This disease is encountered frequently in childhood and presents with progressive atrophy of the unilateral hemisphere, and there are also sustained neurological complications. Owing to uncertain pathogenesis, the most effective way to limit the influence of seizures currently is cerebral hemispherectomy. In this review, we focus on four main lines of pathogenesis: virus infection, antibody-mediated, cell-mediated immunity, and microglia activation. Although one or more antigenic epitopes may give rise to infiltrating T cell responses in RE brain tissue, no exact antigen was confirmed as the definite cause of the disease. On the other hand, the appearance of antibodies related with RE seem to be a secondary pathological process. Synthetic studies have suggested an adaptive immune mechanism mediated by CD8 T cells and an innate immune mechanism mediated by activated microglia and neuroglia. Accordingly, opinions have been raised that immunomodulatory treatments aimed at initial damage to the brain that are induced by cytotoxic CD8 T cell lymphocytes and microglia in the early stage of RE slow down disease progression. However, systematic exploration of the theory behind these therapeutic effects based on multicenter and large sample studies are needed. In addition, dysfunction of the adenosine system, including the main adenosine removing enzyme adenosine kinase and adenosine receptors, has been demonstrated in RE, which might provide a novel therapeutic target for treatment of RE in future.
PubMed: 33294146
DOI: 10.1177/2040622320971413