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Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 16, 2016: Neuro Endocrinology Letters
Marina Aberastury, Betina Comas, María García, Ana Besocke, Carlos Ciraolo, Guillermo Agosta, Walter Silva
Epilepsy surgery in children with refractory epilepsy is one of the most effective methods to control seizures. The proper selection and assessment of surgery candidates is critical for surgical treatment to be adequately effective and safe. The purpose of this article is to describe our experience with 43 consecutive pediatric patients that underwent epilepsy surgery for refractory epilepsy between September 2005 and May 2014. Effectiveness, safety, and prognostic factors were analyzed. The median age was 12 years old at the time of surgery and 4...
October 1, 2016: Archivos Argentinos de Pediatría
Rolla Shbarou
The management of early-onset, genetically determined epilepsies is often challenging. First-line anti-epileptic drugs (AEDs) often include phenobarbital, phenytoin, oxcarbazepine, carbamazepine, clonazepam, levetiracetam, and valproic acid. Combinations of medications are used in these patients with often intractable seizures, and they include topiramate, clobazam, felbamate, lacosamide, lamotrigine, rufinamide, vigabatrin, ACTH, oral steroids, and the ketogenic diet. Vagus nerve stimulator therapy offers some relief in selected patients...
October 2016: Current Treatment Options in Neurology
Tami Katzir, Joanna A Christodoulou, Stella de Bode
OBJECTIVE: We investigated reading skills in individuals who have undergone left cerebral hemispherectomy and in readers with developmental dyslexia to understand diverse characteristics contributing to reading difficulty. Although dyslexia is a developmental disorder, left hemispherectomy requires that patients (re)establish the language process needed to perform the language-based tasks in the nondominant (right) hemisphere to become readers. METHODS: Participants with developmental dyslexia (DD; n = 11) and participants who had undergone left hemispherectomy (HEMI; n = 11) were matched on age and gender, and were compared on timed and untimed measures of single word and pseudo-word reading...
October 2016: Epilepsia
Wei Xiao, Wenya Fu, Tianlong Wang, Lei Zhao
Hemispherectomy is an established surgical procedure to treat medically refractory epilepsy caused by diffuse hemispheric diseases. The most common complication of hemispherectomy is intraoperative bleeding. Perioperative allogeneic blood transfusion increases mortality and morbidity in pediatric patients. Etiologies of massive blood loss during hemispherectomy include intraoperative diffuse vascular damage, antileptic drugs induced coagulation dysfunction, hyperfibrinolysis and dilutional coagulopathy. Great efforts should be made to minimize the need of blood transfusion...
September 2016: Journal of Clinical Anesthesia
Carrie R Muh
The use of epilepsy surgery in various medically resistant epilepsies is well established. For patients with intractable pediatric epilepsy, the role of intracranial electrodes, resective surgery, hemispherectomy, corpus callosotomy, neurostimulation, and multiple subpial transections continues to be very effective in select cases. Newer treatment and diagnostic methods include laser thermal ablation, minimally invasive surgeries, stereo electroencephalography, electrocorticography, and other emerging techniques...
May 2016: Seminars in Pediatric Neurology
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
Michael McGinity, Nicholas Andrade, Kameel Karkar, Jean-Louis Caron, Charles Szabó, Alexander Papanastassiou
BACKGROUND: Status epilepticus (SE) is a medical emergency, as deleterious long-term effects are well known. Medically induced burst suppression is often required if first-line and second-line treatments fail. Surgical intervention can be considered in some patients after prolonged treatment failure of medically induced coma. Multiple surgical options for terminating SE have been demonstrated in the literature, with only 2 reports including hemispherectomy in adults. CASE DESCRIPTION: We present 2 cases of adults with refractory SE who failed more conservative medical/surgical treatment but responded to functional hemispherectomy...
September 2016: World Neurosurgery
PrastiyaIndra Gunawan, Lusiana Lusiana, Darto Saharso
Objective Proteus syndrome is a rare overgrowth disorder including bone, soft tissue, and skin. Central nervous system manifestations were reported in about 40% of the patients including hemimegalencephaly and the resultant hemicranial hyperplasia, convulsions and mental deficiency. We report a 1-month-old male baby referred to Pediatric Neurology Clinic Soetomo Hospital, Surabaya, Indonesia in 2014 presented recurrent seizures since birth with asymmetric dysmorphic face with the right side larger than the left, subcutaneous mass and linear nevi...
2016: Iranian Journal of Child Neurology
Jo Lebeer
BACKGROUND: The theory of Structural Cognitive Modifiability and Mediated Learning Experience of Reuven Feuerstein states that individuals with brain impairment, because of congenital or acquired origin, may substantially and structurally improve their cognitive functioning, by a systematic intervention based on a specific, criteria-based type of interaction ("mediated learning"). Three application systems are based on it: a dynamic-interactive assessment of learning capacity and processes of learning, the LPAD (Learning Propensity Assessment Device); a cognitive intervention program called "Instrumental Enrichment Program", which trains cognitive, metacognitive and executive functions; and a program, which is oriented at working in context, Shaping Modifying Environments...
June 18, 2016: NeuroRehabilitation
Jeffrey P Mullin, Pranay Soni, Sungho Lee, Lara Jehi, Ahsan Moosa Naduvil Valappi, William Bingaman, Jorge Gonzalez-Martinez
BACKGROUND: In some cases of refractory epilepsy, hemispherectomy is the final invasive treatment option. However, predictors of postoperative hemiparesis in these patients have not been widely studied. OBJECTIVE: To investigate how the volumetric analysis of cerebral peduncles and cerebellar hemispheres in patients who have undergone hemispherectomy may determine prognostic implications for postoperative hemiparesis. METHODS: Twenty-two patients who underwent hemispherectomy at our institution were retrospectively included...
September 2016: Neurosurgery
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
John D Rolston, Dario J Englot, Robert C Knowlton, Edward F Chang
Epilepsy surgery is under-utilized, but recent studies reach conflicting conclusions regarding whether epilepsy surgery rates are currently declining, increasing, or remaining steady. However, data in these prior studies are biased toward high-volume epilepsy centers, or originate from sources that do not disaggregate various procedure types. All major epilepsy surgery procedures were extracted from the Centers for Medicare and Medicaid Services Part B National Summary Data File and the American College of Surgeons National Surgical Quality Improvement Program...
August 2016: Epilepsy Research
Loraine Georgy, Alessia Celeghin, Carlo A Marzi, Marco Tamietto, Alain Ptito
Patients with cortical blindness following a lesion to the primary visual cortex (V1) may retain nonconscious visual abilities (blindsight). One intriguing, though largely unexplored question, is whether nonconscious vision in the blind hemifield of hemianopic patients can be sensitive to higher-order perceptual organization, and which V1-independent structure underlies such effect. To answer this question, we tested two rare hemianopic patients who had undergone hemispherectomy, and in whom the only post-chiasmatic visual structure left intact in the same side of the otherwise damaged hemisphere was the superior colliculus (SC)...
August 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Jian Guan, Michael Karsy, Katrina Ducis, Robert J Bollo
Pediatric epilepsy is a debilitating condition that impacts millions of patients throughout the world. Approximately 20-30% of children with recurrent seizures have drug-resistant epilepsy (DRE). For these patients, surgery offers the possibility of not just seizure freedom but significantly improved neurocognitive and behavioral outcomes. The spectrum of surgical options is vast, ranging from outpatient procedures such as vagus nerve stimulation to radical interventions including hemispherectomy. The thread connecting all of these interventions is a common goal-seizure freedom, an outcome that can be achieved safely and durably in a large proportion of patients...
April 2016: Translational pediatrics
Frank Besag, Rochelle Caplan, Albert Aldenkamp, David W Dunn, Giuseppe Gobbi, Matti Sillanpää
There are relatively few studies of the behavioural outcome of epilepsy surgery in children that have used standardised behavioural measures before and after the procedure. Those investigations that have used such measures are often on mixed groups with mixed pathology, implying that the numbers, when stratified, are very small. They are also often retrospective. Furthermore, because placebo surgery is generally not an option, the studies have been open and they are usually uncontrolled. The few available data suggest that, although individual children may benefit or deteriorate, there is little overall group effect of temporal or extratemporal surgery on behavioural/psychiatric outcome...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
A I Maraña Pérez, M L Ruiz-Falcó Rojas, V Puertas Martín, J Domínguez Carral, I Carreras Sáez, A Duat Rodríguez, V Sánchez González
INTRODUCTION: Sturge-Weber syndrome is a congenital vascular disorder characterised by facial capillary malformation (port-wine stain) associated with venous and capillary malformations in the brain and eye. Neurological symptoms and alterations in other locations may also be observed. OBJECTIVES: This study describes the clinical and epidemiological characteristics and different treatments in a cohort of patients diagnosed with Sturge-Weber syndrome in a tertiary hospital...
March 7, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Madeline D Schwarz, Menglu Li, Jackie Tsao, Raymond Zhou, Yvonne W Wu, Raman Sankar, Joyce Y Wu, Shaun A Hussain
BACKGROUND: Vigabatrin (VGB) is one of two FDA-approved medications for treatment of infantile spasms. Despite demonstrated efficacy, its use has been curtailed by reports indicating a substantial risk of VGB-associated visual field loss (VAVFL). As these reports have conflicted with our clinical observations in routine practice, we systematically reviewed the experiences of patients treated with VGB at UCLA to estimate the prevalence of clinically apparent VAVFL. METHODS: Patients with video-EEG-confirmed infantile spasms evaluated at our center between February 2007 and February 2014 were retrospectively identified...
April 2016: Epilepsy & Behavior: E&B
Monique M J van Schooneveld, Kees P J Braun, Peter C van Rijen, Onno van Nieuwenhuizen, Aag Jennekens-Schinkel
PURPOSE: To evaluate cognition, behavior, daily functioning and health-related quality of life (HrQoL) five years to more than a decade after hemispherectomy (HE) in childhood. METHODS: This countrywide Dutch cohort study of 31 patients, who underwent HE between 1994 and 2009, included a semi-structured interview with parents, an assessment of cognition, and screening of behavioral problems and HrQoL. RESULTS: Twenty-two school-age children and young adults [median age 13...
May 2016: European Journal of Paediatric Neurology: EJPN
Khalid Alqadi, Ram Sankaraneni, Ursula Thome, Prakash Kotagal
BACKGROUND: Hypermotor seizures (HMSs) consist of complex movements involving proximal segments of the limbs and trunk that appear violent and inappropriate for the situation. METHODS: We analyzed hypermotor seizure videos in seizure-free patients (Engel class I) following resective epilepsy surgery. After completion of video analysis, we reviewed EEG and neuroimaging data. RESULTS: Search of our epilepsy surgery database yielded 116 patients classified as having hypermotor seizures between 1996 and 2013...
January 2016: Epilepsy & Behavior: E&B
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