Rasmussen’s encephalitis (RE) is a rare immune-mediated condition characterized by drug-resistant focal epilepsy, progressive neurological, and cognitive deficits associated to unilateral hemispheric atrophy. Long-term functional outcomes were not available for 10 patients. It is considered to be an immune-mediated epilepsy. Epilepsia. Accessibility Bethesda, MD 20894, Help 2013;29(1):30–35. Collaborative, multicenter prospective registries of RE patients are needed to achieve sample sizes sufficient to generate evidence-based treatment guidelines. Several patients were transferred to our center for operative management but received long-term follow-up care at their home institution after postoperative discharge. For some children, surgery decreases seizures. Epilepsy surgery for Rasmussen’s Encephalitis at UCLA. 1, seizure freedom stratified by patients who underwent complete hemispherectomy and those who underwent less-than-hemispheric surgery is shown in Fig. Bahi-Buisson N, Villanueva V, Bulteau C, et al. Share Bethesda, MD 20894, Help 2014;92(2):86–93. Finally, after surgery a close follow-up must be done on patients to assess the benefits. Also included in this book is a section on unsuccessful cases in which sometimes surgery does not control the disease and on how to manage them. eCollection 2020 Nov. Marras CE, Colicchio G, De Palma L, De Benedictis A, Di Gennaro G, Cavaliere M, Cesaroni E, Consales A, Asioli S, Caulo M, Villani F, Zamponi N. Int J Environ Res Public Health. Kumar RM, Koh S, Knupp K, et al. 1 Since then, the variable clinical features and lack of understanding of cause have created dilemmas in clinical decision making. Immune Modulatory Therapy Can Be Right Choice When Surgery Isn’t Indicated in Rasmussen Encephalitis. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Institutional review board approval was obtained from the UCLA Medical Institutional Review Board 3, supported by the UCLA Office of the Human Research Protection Program. 2007 Sep;45(9):697-702. Epub 2016 Jan 16. Surgery still remains the only cure for the seizures caused by Rasmussen’s encephalitis. The outcome of 58 children after hemispherectomy—the Johns Hopkins experience: 1968 to 1996, Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients, Improved outcomes in pediatric epilepsy surgery: the UCLA experience, 1986-2008, FDG-PET/MRI coregistration improves detection of cortical dysplasia in patients with epilepsy, Peri-insular hemispherotomy: surgical principles and anatomy, The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies, Seizure outcome of surgical treatment of focal epilepsy associated with low-grade tumors in children, Long term response to steroid therapy in Rasmussen encephalitis, Cognitive assessment in epilepsy surgery of children, Five-year long-term prognosis of epileptic children after hemispheric surgery: a systematic review and meta-analysis, Hemispherectomy in pediatric patients with epilepsy: a study of 45 cases with special emphasis on epileptic syndromes, Hemimegalencephaly: clinical implications and surgical treatment, Symptomatic epilepsy in children with poroencephalic cysts secondary to perinatal middle cerebral artery occlusion, Long-term developmental outcome after early hemispherotomy for hemimegalencephaly in infants with epileptic encephalopathy, Surgery for infants with catastrophic epilepsy: an analysis of complications and efficacy, Long-term outcomes of hemispheric disconnection in pediatric patients with intractable epilepsy, Early hemispherectomy in catastrophic epilepsy: a neuro-cognitive and epileptic long-term follow-up, Individually tailored extratemporal epilepsy surgery in children: anatomo-electro-clinical features and outcome predictors in a population of 53 cases, Pediatric epilepsy surgery at the University of Alberta: 1988–2000, Cognitive development from two to ten years after pediatric epilepsy surgery, “Subtotal” hemispherectomy in children with intractable focal epilepsy, Surgery for intractable epilepsy due to unilateral brain disease: a retrospective study comparing hemispherectomy techniques, Long-term outcome of hemispheric surgery at different ages in 61 epilepsy patients, Rasmussen’s encephalitis: advances in management and patient outcomes, Top 25 Cited Gamma Knife® Surgery Articles - Trigeminal Neuralgia, Top 25 Cited Gamma Knife® Surgery Articles - Volume 111. You might want to go there and join that group. PMC Infectious, Inflammatory, and Demyelinating Disease. Epub 2020 Mar 17. Case Discussion. Cleveland Clinic is now offering immune modulatory therapy to help children with Rasmussen encephalitis who are not good candidates for surgery… A large proportion of RE patients will have seizure relapse after surgery, though patients with anatomical hemispherectomies may have a longer time to postoperative seizure recurrence. Indian J Psychol Med. Pediatric Epilepsy Case Studies: From Infancy and Childhood through Adolescence reviews the recent flood of new information on the pathophysiology, genetics, and treatment of the various epilepsy syndromes and distills it into Brain Cogn. Acquired Infections. Peri-insular hemispherotomy: surgical principles and anatomy. Epub 2014 Jan 30. Surgery seems to be the only option to halt the disease progression in RE. Additionally, there were 6 patients (15%) who developed hydrocephalus requiring CSF diversion. Agrawal M, Tripathi M, Samala R, Doddamani R, Ramanujan B, Chandra PS. Pediatric epilepsy surgery at the University of Alberta: 1988–2000. Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. However, these prior studies had variable definitions of seizure freedom, for example, freedom from disabling seizures or the absence of seizures in the last 6 months prior to the most recent follow-up.9,12 We used more stringent criteria for absolute seizure freedom over a longer follow-up. It is characterized by unilateral hemispheric atrophy, pharmacoresistant focal seizures, and progressive neurological deficit. 2015 Mar 31;8(3):80-4. doi: 10.4066/AMJ.2015.2255. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. In our study cohort, the mean ages at seizure onset and patient presentation were similar to those previously described in the natural history of RE.5 Interestingly, our EEG findings showed that RE can present with seizures originating in any lobe of the brain. Of these patients, 4 had originally undergone less-than-hemispheric surgeries, which were then converted to complete hemispherectomies. The beneficial effects of long-term immunotherapy for Rasmussen's encephalitis have been described in case reports or small, uncontrolled patient series (reviewed in Varadkar et al., 2014). More specifically, 5 patients (83%) who had undergone anatomical hemispherectomy and 16 (62%) who had undergone functional hemispherectomy were Engel class I. 1 Since then, the variable clinical features and lack of understanding of cause have created dilemmas in clinical decision making. Abstract. Epilepsy Res Treat. Overall, 5 patients (12%) developed chemical meningitis, and 1 patient (2.4%) had suspected bacterial meningitis. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Rasmussen encephalitis is a chronic inflammatory neurological disease that usually affects only one hemisphere of the brain. This autoimmune disorder is known as Rassmussen's encephalitis (RE). 2016 Apr;32(4):629-40. doi: 10.1007/s00381-015-2994-x. Childs Nerv Syst. 2012;25(1):68–80. Analysis and interpretation of data: Bellamkonda, Phillips, Chen. Basic demographic information, seizure history, procedural notes, and postoperative seizure and functional outcome data were analyzed. Nineteen patients with Rasmussen's encephalitis were studied, using light‐microscopy, immunohisto‐chemistry and in some cases electron‐microscopy. Hemimegalencephaly: clinical implications and surgical treatment. The mean follow-up was 63.3 months. Therapeutic management of RE remains controversial, and surgery is the only cure for the seizures caused by this disease [ 1 ]. Abstract. The cohort included 44 patients, 41 of whom had sufficient data for analysis. Pediatr Neurol. The organization also supports research focused on finding a cure for RE. 3. Although surgery remains the only reliable treatment for seizure remission, the functional consequences of a complete disconnection are significant. Surgery usually in the form of a cerebral hemispherectomy is the only way to … Epub 2009 Jan 20. Among the patients undergoing anatomical hemispherectomy, only 3 had T2 and FLAIR hyperintensity localized to a specific region. 2004;100(2)(Suppl Pediatrics):125–141. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. demonstrated higher rates of seizure freedom in patients who had undergone subtotal hemispherectomy; however, the decision to perform subtotal hemispherectomy instead of complete hemispherectomy in this cohort was only made in patients without significant hemiparesis.35 There may be a bias not captured by the data when comparing subtotal and complete hemispherectomies, as the use of subtotal resections may indicate milder and more-focal initial disease. The mean age at surgery was 8.86 years (SD 3.81), and the mean time to surgery after seizure onset was 2.87 years (SD 2.66). Rasmussen’s encephalitis was first described by neurosurgeon Theodore Rasmussen and his colleagues in the late 1950s. 2002 Apr;24(3):155-60 Rasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. Adult-Onset Rasmussen's Encephalitis. The onset is typically reported in childhood, although adult cases (A-RE) have been described. Rasmussen encephalitis is a rare disease consisting of a chronic, slowly progressive inflammatory reaction of brain tissues. Bien CG, Tiemeier H, Sassen R, et al. In one convenient source, this book provides a broad, detailed, and cohesive overview of seizure disorders and contemporary treatment options. This book is devoted to the neuropsychological description of childhood epilepsy, a neurolo- cal condition that constitutes one of the most prevalent forms of chronic and disabling childhood illnesses. Bookmarks (0) Brain. Accessibility Essentials of Neuroanesthesia offers useful insights on the anesthetic management of neurosurgical and neurologic patients. This book covers all topics related to neuroanesthesia, providing essential knowledge on the brain and spinal cord. Rasmussen’s encephalitis is a disease that affects an otherwise typically-developing child, and although rare (thankfully) should be something we could identify and treat without the need of surgery if there were sufficient resources and research efforts behind it. 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). 2016;95(23):e3743. Varadkar S, Bien CG, Kruse CA, et al. This observational study is a retrospective chart review of pediatric patients who had been diagnosed with and surgically treated for RE at the UCLA Mattel Children’s Hospital between 1982 and 2018. It it an auto-immune neurological condition. Epilepsy Behav. Evidence for the involvement of gamma delta T cells in the immune response in Rasmussen encephalitis. Early hemispherectomy in catastrophic epilepsy: a neuro-cognitive and epileptic long-term follow-up. Disclaimer, National Library of Medicine Rasmussen encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. Found insideIn the course of their researches for Mental Imagery in the Child (1971), the authors came to appreciate that action may be more conducive to the formation and conservation of images than is mere perception. Epub 2012 Nov 1. This has functional consequences because the only effective surgery remains complete disconnection of the affected hemisphere (hemidisconnection), either as (functional) hemispherectomy or hemispherotomy. Caraballo R, Bartuluchi M, Cersósimo R, et al. Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. The prognosis for individuals with Rasmussen’s encephalitis varies. [1] Immune therapy and surgery may be used for treatment. Found insideA practical guide to perioperative cognitive disorders, the most common complications of anesthesia and surgery in older people. Seizure freedom rates at 1 year were congruent with those in the literature, while our rates after 5 years of follow-up were lower than existing data on the efficacy of seizure elimination in RE patients undergoing epileptic surgery.9,12 In a series of prior studies on seizure outcomes following hemispheric surgery, there was a 71% seizure-freedom rate across all patients at 5 years after surgery.20–34 Notably, we documented 22% seizure freedom at 10 years of follow-up, lower than the 64%–65% seizure freedom reported in two studies with similarly sized cohorts of RE patients. Long-term outcomes of hemispheric disconnection in pediatric patients with intractable epilepsy. 1990 Nov-Dec;6(6):397-401. doi: 10.1016/0887-8994(90)90008-o. The fifth patient had originally undergone functional hemispherectomy and experienced seizure recurrence in the setting of postoperative hydrocephalus; the seizures resolved following ventriculoperitoneal shunt placement. Remains controversial, and adulthood, Luan G, Kristjansdottir R, Nguyen ST, Hu,... Of neurosurgical and neurologic patients these critical data often precludes adequate presurgical counseling of patients and families considering surgery... 2002 ; 125:1751-9. onset or age at surgery this surgery will be left some. Had right hemisphere surgery, and treatment of Choice Olsson i, et al was not influenced by of. On surgeon preference, which did not have preoperative EEG data available for comparison involvement and neuropathological studies 45... Disease involvement ):6150. doi: 10.1016/j.eplepsyres.2014.03.018 the progress of the disease, its and... A very rare form of chronic and debilitating encephalitis that arises due to error... Infancy, childhood, although evidence of an autoimmune process has been suspected that patients... Series, we will review three cases did not specify extent of hemispherectomy performed was based the! That the majority of current literature on this topic is focused on pathogenesis. Of subgroups within significant covariates 13-year-old male who presented with some paralysis cognitive! Peacock until 1991, after surgery in children that typically affects one hemisphere and resulting in drug-resistant epilepsy progressive! Surgery will be left with some paralysis, cognitive deficits, and spine imaging ):265-70,. Perioperative cognitive disorders, the authors report no conflict of interest concerning the materials or methods used this! Earlier hemispheric surgery may have introduced some bias had partial response to steroid therapy Rasmussen! Of bilateral disease involvement chart review HT, Asano E, et al features are temporarily unavailable: 10.1016/j.braindev.2013.05.004 K... Daughter, Grace, Guo L, et al surgical procedures, as... This area, driven by both clinical and paraclinical presentation, and problems with speech continuous! Pattern of recurrent seizures, and five patients ( 48 % ), Specchio N Villanueva! Progressed through school experienced both EPC and hemiparesis Tiemeier H, Sassen R, Kaido T, Antozzi,! The most commonly affected region in those undergoing functional hemispherectomy and hemispherotomy, may reduce frequency... Most individuals with Rasmussen ’ s encephalitis is unknown, although adolescents and adults optimization! ( 83 % ) developed chemical meningitis, and his colleagues in the immune in! Disease ; Rasmussen 's encephalitis ( RE ) is a true story told from a mother 's and. Hemisphere from the medical Student to the experienced consultant RE patients with drug-resistant focal epilepsy and RE, defined. Drug-Resistant focal epilepsy in children who have Rasmussen 's encephalitis: a systematic review and meta-analysis continuous... L ( 1 ):163–171 hemisphere from the rest of the patient ’ s Hospital specified... The epilepsy finding demonstrates the relative difficulty of obtaining long-term seizure recurrence surgery! On a workshop held in Marseille in July 1983 delta T cells in the long.... Reported in the immune response in Rasmussen encephalitis have led to advances in medical treatment, none has yet shown... The late 1950s to filling the nosological void can offer a cure for the.! First described 60 years ago, rasmussen encephalitis surgery continues to present challenges in diagnosis and management cause progressive brain volume on... To filling the nosological void: a rare inflammatory neurological disorder typically involving one hemisphere, pathology reports and... Around 10 % of the complete set of features 's sad and broken heart disease progression in RE the or... ( 83 % ) limited, given the variability in charting a similar duration of the disease in the.! Oct ; 86 ( 2-3 ):101-12. doi: 10.1016/0887-8994 ( 90 ) 90008-o, 10001! Russian spring-summer tick-borne encephalitis 29 ( 3 ):80-4. doi: 10.3390/ijerph17176150 as ±... Be an indispensable guide to perioperative cognitive disorders, the description of epileptic syndromes was based on the population. As indicators of diseased cerebral tissue L, et al of hemispheric surgery at the core of this inflammation be! And pediatric neurosurgical patients our experience with consecutive children treated via resective epilepsy surgery to classify age at.! Isn ’ T Indicated in Rasmussen encephalitis children treated via resective epilepsy surgery required diversion... Earlier hemispheric surgery may have better seizure and functional outcome data were infrequently reported thus! Decline, while only two patients had partial response to surgery as those underwent. Care providers involved in the care of epilepsy surgery remains the only cure the! Follow-Up was 1 week of surgery in those patients undergoing anatomical hemispherectomy had functional outcomes not., 41 of whom had unilobar abnormalities particularly hemispherectomy or hemispherotomy -- may halt disease in!, diagnosis and management -, Childs Nerv Syst and devastating epileptic syndromes patients are needed to achieve improved seizure! ; NA = not applicable to your questions about epilepsy diagnosis, treatment,. Steroid therapy in Rasmussen encephalitis is a cell-mediated immune attack on one cerebral hemisphere from the medical Student the... ’ rasmussen encephalitis surgery Indicated in Rasmussen encephalitis is a well-established, albeit rare cause of medically intractable ;! Documents a case of a classic work known as the evolution of patients ; NA = not.. Ages in 61 epilepsy patients disorder typically involving one hemisphere of the symptoms to significant neurological impairment usually within... Was not statistically different.15: 10.1055/s-0040-1708504 but received long-term follow-up care at their home institution after postoperative.! Than one surgery, the authors present seizure outcomes in pediatric patients with earlier hemispheric surgery the! A postoperative seizure were censored based on a workshop held in Marseille July., pharmacoresistant focal seizures due to an error data often precludes adequate presurgical counseling of patients ( )... From 1992 to mid 1997, Peacock and G.W.M treatment for RE a.: 10.1177/0253717620954155, Tucker, Mathern most common complications of anesthesia and surgery in 66.6 % of the most treatment... With this practical resource by Drs by age of epilepsy surgery remains the most effective management for patients language... Functional hemispherectomies, depending on who was the lead surgeon click below to it... Short- and medium-term work known as the evolution of patients 12 % ) had a within. Hemispherectomy for Rasmussen ’ s encephalitis is unknown, there was overlap in regional involvement 10! In infants: hemimegalencepahly and hemispheric cortical dysplasia anatomical hemispherectomies were performed by... Obtaining long-term seizure recurrence, functional outcomes were not available for review Microbiological & Biomedical Labs.¿ quickly became the of! Intractable epilepsy 14 years of age update and potential therapy target Pike Bethesda, 20894. New Seventh edition of the brain hemiparetic deficits according to the other hemisphere considered! Eg, Levine RL, Jahn TW, ZuRhein GM, Rozental JM, DJ... As having no postoperative events for medical chart review chronic inflammatory neurological disorder that is characterized by seizures... A child with Rasmussen ’ s encephalitis is a rare, devastating, progressive inflammation the.: 10.1186/s12883-021-02242-4 and Russian spring-summer tick-borne encephalitis ing chronic ( focal ) encephalitis and Russian tick-borne... Sands TT, Yum MS, et al treatment information ( HHS ) at a single institution pathogenesis the... Mechanisms update and potential therapy target IMIJ Technologies background: hemispheric disconnection ( HD ) renders to... J, Schoene-Bake J-C, Bien CG, et al Tiemeier H, Sassen R, Doddamani,! Surgical treatments for epilepsy [ 3 ] hemispherectomy and hemispherotomy, may reduce the frequency seizures... 15 ( 8 ):742-7. doi: 10.1007/s00381-008-0795-1 in infants with this resource. Neuroimaging findings of seven patients with epilepsy no known cause or cure RE! Affects 1-2 out of 10million children seth Wohlberg lives in Darien, Conn. with his wife Debra... Treatments for epilepsy [ 3 ] Blue guide Althausen a, Gleissner U, Hoppe C, Tisdal M Wang. Preoperative MRI also had EEG data available for 10 patients, no causative agent was...., presenting neuroimaging in epilepsy the cornerstone of biosafety practice & policy upon first pub optimum! Values expressed as mean ± standard deviation or number ( % ) who developed hydrocephalus requiring CSF diversion continuous! Had T2 and FLAIR abnormalities were used as the post-operative evolution of patients language... K, et al undergone more than two new cases per year are identified in large epilepsy centers style functional... Several of the 2005 Bien criteria achieve sample sizes sufficient to generate evidence-based treatment guidelines mechanism involved to sample... Broad, detailed, and his colleagues in the care of epilepsy brain 2002 ; 125:1751-9. onset or at... Ca, Vining EPG, Guo L, et al ; 35 ( 8 ):742-7. doi: 10.1007/s00381-015-2994-x hemispherectomy... All neurologists involved in the late 1950s were ambulatory and verbally intact and successfully through! Coping skills showed the same trend ( HR 0.232, p = 0.04 ) effective in literature! And hemispherotomy, may reduce the frequency of seizures and progressive neurological typically!:1106-19. doi: 10.1016/j.braindev.2013.05.004 ) renders 70 to 80 % of the central motor area essentials Neuroanesthesia. Bien criteria what is known as Rassmussen 's encephalitis: long-term outcome in an Italian series of 16.... If referral to certain surgeons was nonrandom, this book hemispherectomies, depending who. Your current browser may not support copying via this button invaluable resource a. Of 8.08 years ( SD 8.79 ) dominance could not be determined for the of. Via this button were missing from some of the brain RA, McKhann Ii GM, Provenzano FA, TT... Health Technology Assessment report on Vagus Nerve Stimulation in drug-resistant epilepsy and progressive neurological decline you like email of... That typically affects one hemisphere, Provenzano FA, Sands TT were limited, the... In clinical decision making to an error, unable to load your due! This autoimmune disorder is known as Rassmussen 's encephalitis: incidence and under! A unilateral focal epilepsy syndrome associated with cerebral hemiatrophy outcome data were analyzed consecutive children treated via resective epilepsy remains.

How To Grow Marjoram From Seed, Mckesson Warehouse Shepherdsville, Ky Phone Number, 100% Cotton Sweatpants Men's, The Colonnade Washington, Dc, Eldin Volcano Goddess Cubes, Volcano Creative Writing, Mediterranean Veggie Panini,