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The Lancet. Neurology Feb 2014Rasmussen's encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and... (Review)
Review
Rasmussen's encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. Neuropathological and immunological studies support the notion that Rasmussen's encephalitis is probably driven by a T-cell response to one or more antigenic epitopes, with potential additional contribution by autoantibodies. Careful analysis of the association between histopathology and clinical presentation suggests that initial damage to the brain is mediated by T cells and microglia, suggesting a window for treatment if Rasmussen's encephalitis can be diagnosed early. Advances in neuroimaging suggest that progression of the inflammatory process seen with MRI might be a good biomarker in Rasmussen's encephalitis. For many patients, families, and doctors, choosing the right time to move from medical management to surgery is a real therapeutic dilemma. Cerebral hemispherectomy remains the only cure for seizures, but there are inevitable functional compromises. Decisions of whether or when surgery should be undertaken are challenging in the absence of a dense neurological deficit, and vary by institutional experience. Further, the optimum time for surgery, to give the best language and cognitive outcome, is not yet well understood. Immunomodulatory treatments seem to slow rather than halt disease progression in Rasmussen's encephalitis, without changing the eventual outcome.
Topics: Encephalitis; Humans
PubMed: 24457189
DOI: 10.1016/S1474-4422(13)70260-6 -
BMJ Clinical Evidence Feb 2012About 3% of people will be diagnosed with epilepsy during their lifetime, but about 70% of people with epilepsy eventually go into remission. (Review)
Review
INTRODUCTION
About 3% of people will be diagnosed with epilepsy during their lifetime, but about 70% of people with epilepsy eventually go into remission.
METHODS AND OUTCOMES
We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of monotherapy in newly diagnosed generalised epilepsy (tonic clonic type)? What are the effects of additional treatments in people with drug-resistant generalised epilepsy? What are the effects of surgery in people with drug-resistant generalised epilepsy? We searched: Medline, Embase, The Cochrane Library, and other important databases up to August 2011 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA).
RESULTS
We found 8 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions.
CONCLUSIONS
In this systematic review we present information relating to the effectiveness and safety of the following interventions: monotherapy using carbamazepine, gabapentin, lamotrigine, levetiracetam, phenobarbital, phenytoin, sodium valproate, or topiramate; addition of second-line drugs (lamotrigine or levetiracetam) for drug-resistant epilepsy; and hemispherectomy for drug-resistant epilepsy.
Topics: Anticonvulsants; Carbamazepine; Epilepsy; Epilepsy, Generalized; Humans; Incidence; Phenytoin; Remission Induction; Valproic Acid
PubMed: 22348419
DOI: No ID Found -
Journal of Epilepsy Research Jun 2018Hemispherectomy constitutes an established surgical method in the management of patients with medically intractable epilepsy secondary to severe unilateral hemisphere... (Review)
Review
Hemispherectomy constitutes an established surgical method in the management of patients with medically intractable epilepsy secondary to severe unilateral hemisphere damage. As opposed to focal resections, in hemispherotomies, the entire hemisphere is disconnected from the remaining nervous system, including the functional regions and fibers. Despite this, hemispherotomy is recommended for certain indications, and has good functional and epilepsy outcomes. Here we describe the indications, epilepsy outcomes, and surgical techniques for several hemispheric surgeries.
PubMed: 30090755
DOI: 10.14581/jer.18001 -
BMJ Clinical Evidence Jun 2010About 3% of people will be diagnosed with epilepsy during their lifetime, but about 70% of people with epilepsy eventually go into remission. (Review)
Review
INTRODUCTION
About 3% of people will be diagnosed with epilepsy during their lifetime, but about 70% of people with epilepsy eventually go into remission.
METHODS AND OUTCOMES
We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of monotherapy in newly diagnosed generalised epilepsy (tonic clonic type)? What are the effects of additional treatments in people with drug-resistant generalised epilepsy? What are the effects of surgery in people with drug-resistant generalised epilepsy? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2009 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA).
RESULTS
We found eight systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions.
CONCLUSIONS
In this systematic review we present information relating to the effectiveness and safety of the following interventions: monotherapy using carbamazepine, gabapentin, lamotrigine, levetiracetam, phenobarbital, phenytoin, sodium valproate, or topiramate; addition of second-line drugs (lamotrigine or levetiracetam) for drug-resistant epilepsy; and hemispherectomy for drug-resistant epilepsy.
Topics: Anticonvulsants; Carbamazepine; Epilepsy; Epilepsy, Generalized; Humans; Incidence; Phenytoin; Remission Induction; Valproic Acid
PubMed: 21418687
DOI: No ID Found -
Quantitative Imaging in Medicine and... May 2020
PubMed: 32489942
DOI: 10.21037/qims.2020.03.17 -
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 -
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 -
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 -
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