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Wrench J.M.,University of Melbourne | Matsumoto R.,Kyoto University | Inoue Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Wilson S.J.,University of Melbourne
Epilepsy and Behavior | Year: 2011

The accurate prediction of individual outcomes after epilepsy surgery represents a key challenge facing clinicians. It requires a precise understanding of surgical candidacy and the optimal timing of surgery to maximize a range of outcomes, including medical, psychosocial, cognitive, and psychiatric outcomes. We promote careful consideration of how epilepsy has affected an individual's developmental trajectory as key to constructing more differentiated profiles of postsurgical risk or resilience across multiple outcome measures. This life span approach conceives surgery as a crucial "turning point" in an individual's development from which varied outcome trajectories may follow. This helps clinicians understand the expectations patients and families bring to surgery, and emphasizes the interplay of factors that determine a patient's outcome. It also promotes comprehensive, longitudinal assessment of outcome using data analytical techniques that capture individual differences and identify subgroups with similar trajectories. An ongoing challenge facing clinicians is the development of an outcome classification system that incorporates outcomes other than seizures. We illustrate two emerging areas of research shaping how we define surgical candidacy and predict outcome: (1) using cortico-cortical evoked potentials to identify pathways of seizure propagation and cortico-cortical networks mediating cortical functions, and (2) predicting postoperative depression using a model that incorporates psychosocial and neurobiological factors. The latter research points to the importance of routine follow-up and postoperative psychosocial rehabilitation, particularly in patients deemed at "high risk" for poor outcomes so that early treatment interventions can be implemented. Significantly more research is needed to characterize those patients with poor outcomes who may require re-surgery. © 2011 Elsevier Inc. Source

Shimojima K.,Tokyo Womens Medical University | Imai K.,Shizuoka Institute of Epilepsy and Neurological Disorders | Yamamoto T.,Tokyo Womens Medical University
American Journal of Medical Genetics, Part A | Year: 2010

The recent development of high-throughput analysis for genomic copy numbers has enabled to identify microscopic chromosomal duplications that had never been recognized before. Microarray-based comparative genomic hybridization (aCGH) identified a de novo 2.1-Mb microduplication in the 22q11.22q11.23 region surrounded by low copy repeats (LCRs) LCR22E and LCR22H in a 5-year-old boy with developmental delay, hyperactivity, epilepsy, and distinctive facial features, which were within the wide range of the clinical manifestations of the patients with the same duplication pattern. Fiber-fluorescent in situ hybridization (FISH) analysis confirmed that the duplicated segments were aligned in a tandem configuration. Familial single nucleotide polymorphism (SNP) typing determined that the duplication was derived from paternal interchromosomal non-allelic homologous recombination (NAHR) during the first meiotic process of spermatogenesis. Although no patient with the deletions of the distal 22q11.2 has been reported as showing epilepsy, at least five patients including the presenting patient having the duplication between LCR22E and LCR22G showed epilepsy. Thus, the gain of the genomic copy number of this region may have epileptogenesis. © 2010 Wiley-Liss, Inc. Source

Miya K.,University of Toyama | Takahashi Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Mori H.,University of Toyama
Brain and Development | Year: 2014

The N-methyl- d-aspartate receptor (NMDAR) is involved in normal physiological and pathological states in the brain. Anti-NMDAR encephalitis is characterized by memory deficits, seizures, confusion, and psychological disturbances in males and females of all ages. This type of encephalitis is often associated with ovarian teratoma in young women, but children are less likely to have tumors. Anti-NMDAR encephalitis is a neuroimmune syndrome in patients with autoantibodies recognizing extracellular epitopes of NMDAR, and the autoantibodies attenuate NMDAR function through the internalization of NMDAR. Following the initial symptoms of inflammation, the patients show the various symptoms such as memory loss, confusion, emotional disturbances, psychosis, dyskinesis, decrease in speech intelligibility, and seizures. About half of these patients improved with immunotherapy including high-dose intravenous corticosteroids and intravenous immunoglobulins is administrated to these patients, but the patients who had no improvement with these therapy require further treatments with rituximab or cyclophosphamide. It is necessary to detect anti-NMDAR antibodies at early stages, because the prognosis of these patients may be improved by early treatment. Recovery is slow, and the patients may have some disturbances in their motor function and cognition. The pathologic mechanism underlying the development of anti-NMDAR encephalitis has been elucidated gradually, but the optimal treatment has not yet been clarified. Further studies are required to clarify in detail the mechanism underlying anti-NMDA encephalitis and to develop effective treatments. © 2013 The Japanese Society of Child Neurology. Source

Usui N.,Shizuoka Institute of Epilepsy and Neurological Disorders
Neurologia Medico-Chirurgica | Year: 2016

This article reviews the current topics in the field of epilepsy surgery. Each type of epilepsy is associated with a different set of questions and goals. In mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis (HS), postoperative seizure outcome is satisfactory. A recent meta-analysis revealed superior seizure outcome after anterior temporal lobectomy compared with selective amygdalohippocampectomy; in terms of cognitive outcome; however, amygdalohippocampectomy may be beneficial. In temporal lobe epilepsy with normal magnetic resonance imaging (MRI), postoperative seizure outcome is not as favorable as it is in MTLE with HS; further improvement of seizure outcome in these cases is necessary. Focal cortical dysplasia is the most common substrate in intractable neocortical epilepsy, especially in children, as well as in MRI-invisible neocortical epilepsy. Postoperative seizure-free outcome is approximately 60-70%; further diagnostic and therapeutic improvement is required. Regarding diagnostic methodology, an important topic currently under discussion is wideband electroencephalogram (EEG) analysis. Although high-frequency oscillations and ictal direct current shifts are considered important markers of epileptogenic zones, the clinical significance of these findings should be clarified further. Regarding alternatives to surgery, neuromodulation therapy can be an option for patients who are not amenable to resective surgery. In addition to vagus nerve stimulation, intracranial stimulation such as responsive neurostimulation or anterior thalamic stimulation is reported to have a modest seizure suppression effect. Postoperative management such as rehabilitation and antiepileptic drug (AED) management is important. It has been reported that postoperative rehabilitation improves postoperative employment status. Pre- and post-operative comprehensive care is mandatory for postoperative improvement of quality of life. © 2016, Japan Neurosurgical Society. All rights reserved. Source

Kubota H.,Shizuoka Institute of Epilepsy and Neurological Disorders | Awaya Y.,Seibo International Catholic Hospital
Epilepsy and Behavior | Year: 2010

The aim of epilepsy treatment is not only to eliminate seizures, but also to improve health-related quality of life (HRQOL). We conducted a postal self-administered survey of HRQOL for Japanese patients using the Quality of Life in Epilepsy inventory (QOLIE-31), Version 1.0, and analyzed factors influencing their quality of life (QOL). Data from 599 analyzable patients were evaluated and a number of factors influencing QOL were identified, including severity and frequency of seizures, seizure control, type of epilepsy, contributing events such as injuries and falls during seizures, number of antiepileptic drugs, employment status, and surgical outcome. These findings suggest that comprehensive management of the patient should be emphasized. Consideration of all the results led to classification of these factors as one of two types: "all or nothing" and "linear." With respect to "all or nothing" factors (e.g. "daytime remarkable seizures"), patients may not be able to improve their QOL unless these factors can be completely controlled. Comparison of each score on the QOLIE-31 subscales with published data revealed that the scores for the subscale Medication Effects were markedly low. © 2010 Elsevier Inc. Source

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