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Yasunori Nagahama, Charuta Joshi, Brian Dlouhy, Angela Y Wu, Taylor J Abel, Gary Baumbach, Hiroto Kawasaki
A 7-year-old previously healthy girl presented with a left-sided focal seizure without impaired consciousness and subsequently developed epilepsia partialis continua. Initial MRI was normal, and the subsequent images only showed a focal T2/FLAIR hyperintense area without cortical atrophy. She was diagnosed with Rasmussen syndrome by pathology and promptly treated with functional hemispherotomy. Rasmussen syndrome is a rare progressive neurological disorder, the only definitive cure for which is hemispheric disconnection...
2017: Epilepsy & Behavior Case Reports
Christine Bulteau, Isabelle Jambaqué, Catherine Chiron, Sebastian Rodrigo, Georg Dorfmüller, Olivier Dulac, Lucie Hertz-Pannier, Marion Noulhiane
The neural networks involved in language recovery following hemispherotomy of the dominant hemisphere after language acquisition in children remain poorly known. Twelve hemispherotomized children (mean age at surgery: 11.3years) with comparable post-operative neuropsychological patterns underwent multi-task language functional MRI. Three of them had recovered from an initial postoperative aphasia i.e., hemispherotomy was performed on the language-dominant hemisphere. Our main results revealed (1) perisylvian activations in all patients after either left or right hemispherotomy; (2) no differences in activations between groups regarding the side of hemispherotomy; (3) additional activations in pre-frontal (3/3) and hippocampal/parahippocampal and occipito-parietal (2/3) areas, when comparing language activation in each of the three subjects with hemispherotomy of the language-dominant hemisphere to the group of 9 non-dominant hemispherotomized patients...
February 22, 2017: Epilepsy & Behavior: E&B
Anna Jeong, Jennifer Strahle, Ananth K Vellimana, David D Limbrick, Matthew D Smyth, Mary Bertrand
OBJECTIVE Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment...
January 2017: Journal of Neurosurgery. Pediatrics
Xiangyu Liu, Taisuke Otsuki, Akio Takahashi, Takanobu Kaido
INTRODUCTION: The authors here present a rare case of a 3-month-old infant with unilateral Sturge-Weber syndrome (SWS) who had excellent seizure control and no aggravation of previous existed neurological deficits after vertical parasagittal hemispherotomy (VPH). To our knowledge, this patient with SWS was the youngest one who received VPH. CASE DESCRIPTION: The use of VPH results in a successful treatment of intractable epilepsy in a patient with seizure onset in early infancy...
2016: SpringerPlus
Tatjana Traub-Weidinger, Philip Weidinger, Gundrun Gröppel, Georgios Karanikas, Wolfgang Wadsak, Gregor Kasprian, Christian Dorfer, Anastasia Dressler, Angelika Muehlebner, Marcus Hacker, Thomas Czech, Martha Feucht
OBJECTIVE The objective of this study was to investigate whether fluorine-18 fluorodeoxyglucose PET ((18)F-FDG PET) can help to predict seizure outcome after hemispherotomy and therefore may be useful in decision making and patient selection. METHODS Children and adolescents less than 18 years of age who underwent (18)F-FDG PET studies during presurgical evaluation prior to hemispherotomy and had follow-up data of at least 12 months after surgery were included. Seizure outcome was classified according to the recommendations of the International League Against Epilepsy...
December 2016: Journal of Neurosurgery. Pediatrics
Gudrun Gröppel, Christian Dorfer, Anastasia Dressler, Angelika Mühlebner, Barbara Porsche, Johann A Hainfellner, Thomas Czech, Martha Feucht
AIM: To study the effect of hemispherotomy on electrical status epilepticus in sleep (ESES) and language development. METHOD: Children with a confirmed diagnosis of ESES prior to surgery and a minimum of 24 months of developmental follow-up data were compared with age-matched controls without ESES. Language quotients (LQs) were calculated before and after surgery. RESULTS: Eleven patients (five females, six males) and 21 controls (11 females, 10 males) were included...
August 25, 2016: Developmental Medicine and Child Neurology
Yuri Nagai, Ayataka Fujimoto, Tohru Okanishi, Hironao Motoi, Sotaro Kanai, Takuya Yokota, Hideo Enoki, Mitsuyo Nishimura, Takamichi Yamamoto
PURPOSE: To perform an epilepsy surgery on a patient with drug resistant epilepsy secondary to bilateral brain malformation. The patient was a 2-year 9-month-old boy who had congenital bilateral multiple abnormalities. He developed a complex partial seizure at 9 months old. Based on the presurgical evaluations, he underwent a right hemispherotomy. RESULTS: Brain MRI revealed congenital bilateral polymicrogyria, right schizencephaly, and corpus callosum agenesis...
2016: Epilepsy & Behavior Case Reports
Gerald F Tuite, Carolyn M Carey, William W Nelson, Scott J Raffa, S Parrish Winesett
Profuse bleeding originating from an injured cerebral sinus can be a harrowing experience for any surgeon, particularly during an operation on a young child. Common surgical remedies include sinus ligation, primary repair, placement of a hemostatic plug, and patch or venous grafting that may require temporary stenting. In this paper the authors describe the use of a contoured bioresorbable plate to hold a hemostatic plug in place along a tear in the inferomedial portion of a relatively inaccessible part of the posterior segment of the superior sagittal sinus in an 11-kg infant undergoing hemispherotomy for epilepsy...
October 2016: Journal of Neurosurgery. Pediatrics
Hanna Küpper, Manfred Kudernatsch, Tom Pieper, Samuel Groeschel, Jacques-Donald Tournier, David Raffelt, Peter Winkler, Hans Holthausen, Martin Staudt
Hemidisconnections (i.e. hemispherectomies or hemispherotomies) invariably lead to contralateral hemiparesis. Many patients with a pre-existing hemiparesis, however, experience no deterioration in motor functions, and some can still grasp with their paretic hand after hemidisconnection. The scope of our study was to predict this phenomenon. Hypothesizing that preserved contralateral grasping ability after hemidisconnection can only occur in patients controlling their paretic hands via ipsilateral corticospinal projections already in the preoperative situation, we analysed the asymmetries of the brainstem (by manual magnetic resonance imaging volumetry) and of the structural connectivity of the corticospinal tracts within the brainstem (by magnetic resonance imaging diffusion tractography), assuming that marked hypoplasia or Wallerian degeneration on the lesioned side in patients who can grasp with their paretic hands indicate ipsilateral control...
September 2016: Brain: a Journal of Neurology
A G Melikyan, Yu V Kushel', A N Vorob'ev, N A Arkhipova, V S Sorokin, N V Lemeneva, I A Savin, I N Pronin, A B Kozlova, O A Grinenko, S B Buklina, I A Nagorskaya
INTRODUCTION AND PURPOSE: Hemispherectomy is a recognized option in the treatment of symptomatic forms of intractable focal epilepsy in patients with developmental brain malformations and some acquired lesions of one the hemispheres. The prognosis for an outcome of the technique is important in terms of the indications for surgical treatment. MATERIAL AND METHODS: We described the hemispherectomy technique and its variants and analyzed our own experience of surgery in 40 children...
2016: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
Anneli Ozanne, Cecilia Verdinelli, Ingrid Olsson, Ulla H Graneheim, Kristina Malmgren
Severe childhood epilepsy has an impact on the whole family. For selected children, hemispherotomy is the treatment of choice. The aim of this study was to explore parents' experiences before and after hemispherotomy as reported at a long-term follow-up and their view on received information and support. This was a population-based qualitative descriptive study, using qualitative content analysis of interviews. Seven to eighteen years after hemispherotomy, parents of twenty-one operated children were interviewed about the family life situation, expectations before surgery, and support and information before and after surgery...
July 2016: Epilepsy & Behavior: E&B
Stanislas Lagarde, Nathalie Villeneuve, Agnès Trébuchon, Elsa Kaphan, Anne Lepine, Aileen McGonigal, Agathe Roubertie, Marie-Anne J Barthez, Valérie Trommsdorff, Jérémie Lefranc, Samer Wehbi, Vincent des Portes, Virginie Laguitton, Pierre Quartier, Didier Scavarda, Bernard Giusiano, Mathieu Milh, Christine Bulteau, Fabrice Bartolomei
OBJECTIVE: Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug-resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect...
June 2016: Epilepsia
Daniel T Nilsson, Kristina Malmgren, Roland Flink, Bertil Rydenhag
BACKGROUND: Reports on outcome after multilobar resection (MLR) are scarce and most are retrospective single-centre studies or case studies with few patients. The aim of this study is to present seizure and complication outcomes 2 years after MLR in a prospective population-based series. METHOD: The Swedish National Epilepsy Surgery Registry (SNESUR) provides prospective population-based data on outcome and complications after epilepsy surgery. For this study, we have analysed data on seizure outcome and complications after MLR from the SNESUR between 1990 and 2013...
June 2016: Acta Neurochirurgica
Sophia Varadkar, Martin Tisdall
Investigators from Rothschild Foundation Hospital in Paris report language outcome in children after (left) dominant-hemisphere hemispherotomy for Rasmussen Syndrome.
February 2016: Pediatric neurology briefs
P Sarat Chandra, Manjari Tripathi
No abstract text is available yet for this article.
July 2016: Journal of Neurosurgery. Pediatrics
Megan Holec, Yasunori Nagahama, Christopher Kovach, Charuta Joshi
OBJECTIVE: We present a child with Rasmussen encephalitis and highlight the pitfalls of diagnosis when magnetic resonance imaging (MRI) is negative for atrophy. We review the literature regarding this issue, introduce the FreeSurfer software as a potential means of noninvasive diagnosis, and discuss methods for prompt and definitive treatment. METHODS: In addition to the patient description, we review the English language literature regarding pathologic diagnosis of Rasmussen encephalitis using the key words Rasmussen encephalitis, focal lesions, MRI, atrophy, epilepsia partialis continua and hemiparesis in PubMed...
June 2016: Pediatric Neurology
Caitlin E Hoffman, Ayako Ochi, Orlando Carter Snead, Elysa Widjaja, Cynthia Hawkins, Martin Tisdal, James T Rutka
PURPOSE: Rasmussen's encephalitis (RE) is a hemispheric inflammatory disorder resulting in progressive epilepsy, hemiparesis, and cognitive decline. Controversy surrounds the most effective timing of surgery with respect to language dominance, functional status, and seizure outcome. We describe our experience with RE to inform treatment decisions. METHODS: A retrospective chart review was performed in children diagnosed with RE from 1983 to 2012. RESULTS: Thirteen consecutive cases were identified: six males and seven females with a mean age of 10...
April 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Jessica Save-Pédebos, Charlotte Pinabiaux, Georg Dorfmuller, Sarah Ferrand Sorbets, Olivier Delalande, Isabelle Jambaqué, Christine Bulteau
PURPOSE: Hemispherotomy (H) is the standard treatment used to cure hemispheric epileptic syndromes in childhood. The postoperative linguistic profile involves hemispheric specialization processes and developmental cognitive plasticity. This research concerns pragmatic aspects of language as a tool for communication which involves both linguistic and extralinguistic communication in context. Our aim was to analyze whether any correlation exists with age at surgery and side of surgery on pragmatic skills following H...
February 2016: Epilepsy & Behavior: E&B
K Pradeep, S Sinha, A Mahadevan, J Saini, A Arivazhagan, R D Bharath, P S Bindu, R Jamuna, M B Rao, S Govekar, B V Ravikumar, B A Chandramouli, P Satishchandra
We studied the clinical, electrophysiological, imaging and pathological features of 18 patients with Rasmussen's encephalitis (RE). This descriptive study included 18 patients (six males, 12 females) with RE who were evaluated for demographic and phenotypic details, electroencephalogram (EEG) results, MRI results, pathological features, virological markers and outcome. Radiological staging as per Bien et al. and pathological staging in accordance with Robitaille et al. were performed. Simple partial seizures were the most common initial manifestation...
March 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Erin N Kiehna, Elysa Widjaja, Stephanie Holowka, O Carter Snead, James Drake, Shelly K Weiss, Ayako Ochi, Eric M Thompson, Cristina Go, Hiroshi Otsubo, Elizabeth J Donner, James T Rutka
OBJECT Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients. The authors hypothesized that residual fiber connections between the hemispheres could be the underlying cause of recurrent epilepsy in these patients. Diffusion tensor imaging (DTI) was used to test this hypothesis, and to target residual connections at reoperation using neuronavigation...
April 2016: Journal of Neurosurgery. Pediatrics
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