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2.
No Shinkei Geka ; 51(1): 156-165, 2023 Jan.
Artigo em Japonês | MEDLINE | ID: mdl-36682761

RESUMO

Apart from epilepsy specialists, neurosurgeons should also play an important role for epilepsy treatment in the Japanese healthcare structure. This review focuses on lifestyle guidance and counselling methods for general neurosurgeons to reduce risks of seizures in epilepsy patients, and to maximize patient independence. Reduction of seizure precipitants such as sleep deprivation, emotional stress, fatigue, and alcohol is key to epilepsy practice with appropriate anti-seizure medication. The modular service package epilepsy(MOSES)program may prove useful for patients by increasing awareness of their own seizure precipitants and empower them to manage their individual risks. This article also addresses the topics of practical guidance for counselling in(1)school life;(2)sports and physical activities;(3)pregnancy; and(4)eating habits. Multidimensional collaborations should be recommended to reduce the burden of hectic neurosurgeons.


Assuntos
Epilepsia , Estresse Psicológico , Humanos , Epilepsia/terapia , Estilo de Vida , Aconselhamento , Privação do Sono
3.
J Neurosurg Pediatr ; 28(4): 395-403, 2021 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-34388720

RESUMO

OBJECTIVE: Pediatric epilepsy surgery is known to be effective, but early surgery in infancy is not well characterized. Extensive cortical dysplasia, such as hemimegalencephaly, can cause refractory epilepsy shortly after birth, and early surgical intervention is indicated. However, the complication rate of early pediatric surgery is significant. In this study, the authors assessed the risk-benefit balance of early pediatric epilepsy surgery as relates to developmental outcomes. METHODS: This is a retrospective descriptive study of 75 patients who underwent their first curative epilepsy surgery at an age under 3 years at the authors' institution between 2006 and 2019 and had a minimum 1-year follow-up of seizure and developmental outcomes. Clinical information including surgical complications, seizure outcomes, and developmental quotient (DQ) was collected from medical records. The effects of clinical factors on DQ at 1 year after surgery were evaluated. RESULTS: The median age at surgery was 6 months, peaking at between 3 and 4 months. Operative procedures included 27 cases of hemispherotomy, 19 cases of multilobar surgery, and 29 cases of unilobar surgery. Seizure freedom was achieved in 82.7% of patients at 1 year and in 71.0% of patients at a mean follow-up of 62.8 months. The number of antiseizure medications (ASMs) decreased significantly after surgery, and 19 patients (30.6%) had discontinued their ASMs by the last follow-up. Postoperative complications requiring cerebrospinal fluid (CSF) diversion surgery, such as hydrocephalus and cyst formation, were observed in 13 patients (17.3%). The mean DQ values were 74.2 ± 34.3 preoperatively, 60.3 ± 23.3 at 1 year after surgery, and 53.4 ± 25.1 at the last follow-up. Multiple regression analysis revealed that the 1-year postoperative DQ was significantly influenced by preoperative DQ and postoperative seizure freedom but not by the occurrence of any surgical complication requiring CSF diversion surgery. CONCLUSIONS: Early pediatric epilepsy surgery has an acceptable risk-benefit balance. Seizure control after surgery is important for postoperative development.


Assuntos
Desenvolvimento Infantil , Epilepsia Resistente a Medicamentos/cirurgia , Procedimentos Neurocirúrgicos/métodos , Fatores Etários , Derivações do Líquido Cefalorraquidiano , Pré-Escolar , Feminino , Seguimentos , Hemisferectomia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Medição de Risco , Convulsões/epidemiologia , Convulsões/cirurgia , Resultado do Tratamento
4.
Acta Neurochir (Wien) ; 163(8): 2121-2133, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33990885

RESUMO

BACKGROUND: The genesis of central post-stroke pain (CPSP) is important but difficult to understand. We evaluated the involvement of the thalamic anterior part of the ventral posterolateral nucleus (VPLa) and central lateral nucleus (CL) in the occurrence of CPSP. METHOD: Stereotactic thalamotomy was performed on the posterior part of the ventral lateral nucleus (VLp)-VPLa and CL in 9 patients with CPSP caused by deep-seated intracerebral hemorrhage. Computed tomography (CT) did not reveal definite thalamic lesion in 5 patients but did in 4 patients. Electrophysiological studies of these thalamic nuclei were carried out during the surgery. Anatomical studies using CT were performed in another 20 patients with thalamic hemorrhage who had clear consciousness but had sensory disturbance at onset. RESULTS: Neural activities were preserved and hyperactive and unstable discharges (HUDs) were often recognized along the trajectory in the thalamic VLp-VPLa in 5 patients without thalamic lesion. Surgical modification of this area ameliorated pain, particularly movement-related pain. Neural activities were hypoactive in the other 4 patients with thalamic lesion. However, neural activities were preserved and HUDs were sometimes recognized in the CL. Sensory responses were seen, but at low rate, in the sensory thalamus. Anatomical study showed that the thalamic lesion was obviously smaller in the patients with developing pain in the chronic stage. CONCLUSIONS: Change in neural activities around the cerebrovascular disease lesion in the thalamic VPLa or CL might affect the perception of sensory impulses or sensory processing in those thalamic nuclei, resulting in the genesis of CPSP.


Assuntos
Núcleos Intralaminares do Tálamo , Neuralgia , Acidente Vascular Cerebral , Núcleos Ventrais do Tálamo , Hemorragia Cerebral , Humanos , Neuralgia/etiologia , Acidente Vascular Cerebral/complicações , Núcleos Ventrais do Tálamo/cirurgia
5.
Neuromodulation ; 24(2): 361-372, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32620052

RESUMO

OBJECTIVES: The effects of thalamic stimulation of the anterior part of the ventral posterolateral nucleus (VPLa) for central poststroke pain (CPSP) and the pain-related electrophysiological characteristics of this structure were investigated. MATERIALS AND METHODS: Nine patients with CPSP manifesting as hemibody pain were enrolled. Stereotactic thalamic VPLa stimulation was implemented, and intraoperative electrophysiological studies on hyperactive and unstable discharges (HUDs) and responses to sensory and electrical stimulation were performed in the sensory thalamus. A preoperative somatosensory-evoked potential (SEP) study was carried out in all nine patients and in eight other patients with localized pain. RESULTS: The patients were classified into two groups: a HUD-dominant group (group H, n = 5) and a sensory response-dominant group (group R, n = 4). HUDs were frequently encountered in the thalamic VPLa in the former group. The total number of HUDs and the number along the trajectory to the VPLa in group H were significantly larger than those in group R. The improvements on the pain numeric rating scale in group H were significantly higher than those in group R two years after surgery. The amplitude ratio of the SEP N20s in the ipsilateral to the contralateral side of CVD lesion in the study group was significantly lower than in the localized pain group. CONCLUSIONS: Adequate and stable pain relief with thalamic VPLa stimulation is obtainable in patients with CPSP who exhibit hyperactivity and electrical instability along the trajectory to this nucleus. Both responders and nonresponders were found to have severe dysfunction of the lemniscal system.


Assuntos
Neuralgia , Acidente Vascular Cerebral , Estimulação Elétrica , Humanos , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Tálamo , Núcleos Ventrais do Tálamo
6.
J Neurosurg Pediatr ; 26(5): 543-551, 2020 Aug 07.
Artigo em Inglês | MEDLINE | ID: mdl-32764180

RESUMO

OBJECTIVE: Cognitive risk associated with insular cortex resection is not well understood. The authors reviewed cognitive and developmental outcomes in pediatric patients who underwent resection of the epileptogenic zone involving the insula. METHODS: A review was conducted of 15 patients who underwent resective epilepsy surgery involving the insular cortex for focal cortical dysplasia, with a minimum follow-up of 12 months. The median age at surgery was 5.6 years (range 0.3-13.6 years). Developmental/intelligence quotient (DQ/IQ) scores were evaluated before surgery, within 4 months after surgery, and at 12 months or more after surgery. Repeated measures multivariate ANOVA was used to evaluate the effects on outcomes of the within-subject factor (time) and between-subject factors (resection side, anterior insular resection, seizure control, and antiepileptic drug [AED] reduction). RESULTS: The mean preoperative DQ/IQ score was 60.7 ± 22.8. Left-side resection and anterior insular resection were performed in 9 patients each. Favorable seizure control (International League Against Epilepsy class 1-3) was achieved in 8 patients. Postoperative motor deficits were observed in 9 patients (permanent in 6, transient in 3). Within-subject changes in DQ/IQ were not significantly affected by insular resection (p = 0.13). Postoperative changes in DQ/IQ were not significantly affected by surgical side, anterior insular resection, AED reduction, or seizure outcome. Only verbal function showed no significant changes before and after surgery and no significant effects of within-subject factors. CONCLUSIONS: Resection involving the insula in children with impaired development or intelligence can be performed without significant reduction in DQ/IQ, but carries the risk of postoperative motor deficits.

7.
Childs Nerv Syst ; 36(5): 1043-1048, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31845027

RESUMO

PURPOSE: This study aimed to elucidate the clinical features of water-electrolyte disturbance (WED) as a sequela of hemispherotomy. METHODS: We performed a retrospective chart review to identify the clinical features of diabetes insipidus (DI) as a complication in < 12-month-old patients who underwent hemispherectomy or hemispherotomy for severe epilepsy between 2007 and 2018. Central DI was diagnosed if a patient developed polyuria (urine output > 5 mL/kg/h), abnormally high serum osmolality (> 300 mOsm/kg), high serum sodium level (> 150 mEq/L), either abnormally low urine specific gravity (< 1.005) or low urine osmolality (< 300 mOsm/kg) or both, and effective control of polyuria with arginine vasopressin (AVP). The clinical course of post-hemispherotomy WED, complications other than WED, and seizure outcomes were analyzed. RESULTS: The review identified that 3 of 23 infants developed WED. All patients developed polyuria within 2 days after surgery, with high serum osmolality and hypotonic urine; AVP was effective in treating these symptoms. The clinical course was compatible with central DI. Two patients subsequently developed hyponatremia in a biphasic or triphasic manner. All patients had multiple seizures that were probably related to WED. Two patients developed asymptomatic cerebral sinovenous thrombosis, possibly because of the surgical procedure and dehydration; anticoagulant treatment was provided. All patients were treated for WED for up to 2 months and had no residual pituitary dysfunction. CONCLUSION: Systemic complications other than intracranial ones can occur in patients who have undergone hemispherotomy. Perioperative systemic management of young infants undergoing this procedure should include careful water and electrolyte balance monitoring.


Assuntos
Epilepsia , Hemisferectomia , Desequilíbrio Hidroeletrolítico , Epilepsia/cirurgia , Humanos , Lactente , Estudos Retrospectivos , Água
8.
World Neurosurg ; 117: e349-e361, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29909211

RESUMO

OBJECTIVE: Long-term effectiveness of selective ventralis intermedius nucleus (VIM)-ventralis oralis nucleus (VO) thalamotomy with depth microrecording for the treatment of focal dystonia was evaluated. The optimal thalamic areas for controlling focal dystonia were studied based on the electrophysiologic and anatomic data. METHODS: Stereotactic selective VIM-VO thalamotomy with depth microrecording was carried out in 8 patients with focal arm and hand dystonia and in 1 patient with cervical dystonia. Electrophysiologic data on the lateral part of thalamic VIM were studied in patients with focal dystonia. A very small and narrow therapeutic lesion was formed in the shape of a square on the sagittal plane and of an I, rotated V, Y, or inverse Y on the axial plane in the VIM-VO, which covered the kinesthetic response area topographically related to focal dystonia. Patients with arm and hand dystonia were followed up for 4.7 ± 3.0 years and 1 patient with cervical dystonia was followed up for 18.2 years. RESULTS: Marked improvement of focal dystonia was shown by functional assessment using the Unified Dystonia Rating Scale. Transient dysarthria was recognized in 1 patient. The sequence of body localization of kinesthetic response in the VIM was clearly shown in patients with focal dystonia. Decreases in the amplitude and amplitude ratio of electromyography on the forearm muscles were markedly significant after VIM thalamotomy, but insignificant after VO thalamotomy immediately after VIM thalamotomy. CONCLUSIONS: Marked reduction of electromyographic tonic discharges of focal dystonia was shown after VIM lesioning. Selective VIM-VO thalamotomy showed good and long-term stable effects for focal dystonia.


Assuntos
Distúrbios Distônicos/cirurgia , Técnicas Estereotáxicas , Tálamo/cirurgia , Adulto , Braço , Distúrbios Distônicos/fisiopatologia , Eletromiografia , Feminino , Mãos , Humanos , Masculino , Microeletrodos , Pessoa de Meia-Idade , Resultado do Tratamento , Tremor/fisiopatologia , Tremor/cirurgia , Núcleos Ventrais do Tálamo/cirurgia , Adulto Jovem
9.
World Neurosurg ; 109: e630-e641, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29054781

RESUMO

OBJECTIVE: The minimum and essential thalamic areas for reducing tremor were investigated in cases treated by superselective thalamotomy in the most lateral part of the ventralis intermedius nucleus (mlp-VIM). METHODS: Stereotactic superselective VIM thalamotomy with depth microrecording was performed in 21 patients with essential tremor (ET) and 15 patients with tremor-dominant Parkinson disease (PD). A very small and narrow (axial plane) therapeutic lesion was formed as a square on the sagittal plane and inverse V on the axial plane in the mlp-VIM, which covered the kinesthetic response area topographically related to tremor. Patients with ET were followed up for 4.7 ± 3.0 years and patients with PD for 7.9 ± 3.9 years. RESULTS: Almost complete tremor control was achieved in all patients immediately after surgery and continued for up to 8 years. A few adverse events were recognized but disappeared within 1 month without 1 patient with thalamic hemorrhage. The medial border of the therapeutic lesion was significantly more lateral in both patients with ET and patients with PD than the calculated standard target point and was in patients with PD than in patients with ET. The mean width was only about 2.4 mm. The individual differences of the adequate location of the therapeutic lesion were significantly greater in the ET than in the PD group. CONCLUSIONS: The important area for reducing tremor was small and narrow and was located in the mlp-VIM, where the proprioceptive ascending signals from the tremor-dominant body part are conducted. Superselective thalamotomy in the mlp-VIM was safe and effective for the long-term in patients with ET and PD.


Assuntos
Tremor Essencial/cirurgia , Doença de Parkinson/cirurgia , Tremor/cirurgia , Núcleos Ventrais do Tálamo/cirurgia , Idoso , Feminino , Seguimentos , Humanos , Cinestesia , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Doença de Parkinson/complicações , Propriocepção , Técnicas Estereotáxicas , Tálamo/cirurgia , Resultado do Tratamento , Tremor/etiologia
10.
Neuropathology ; 38(3): 300-304, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29250844

RESUMO

A rosette-forming glioneuronal tumor (RGNT) was initially reported as an infratentorial tumor that comprised both small neurocytic rosettes and astrocytic components. However, a few studies have reported supratentorial RGNTs arising in the cerebral hemispheres. Here, we report an unusual case involving a 9-year-old boy with a supratentorial RGNT who presented with intractable epilepsy and behavioral changes. Brain MRI revealed a well-circumscribed space-occupying lesion with septae in the right inferomedial parietal lobe. Electroencephalography showed multifocal spikes over the right frontal, temporal and parietal regions. The seizure frequency decreased dramatically after tumorectomy. Histopathological examination revealed prominent neurocytic rosette formation appearing with the specific glioneuronal element of a dysembryoplastic neuroepithelial tumor (DNT). Although the pathogenesis has not been elucidated, a supratentorial RGNT presenting with epilepsy may exhibit a rosette component, which is the major feature of this tumor, against the background of a specific glioneuronal element mimicking DNT. However, RGNT arising in regions other than the fourth ventricle is rare, and the pathogenesis of epilepsy due to RGNT has not been fully elucidated. Further clinical and histological studies are required to understand the pathology underlying epilepsy caused by RGNT.


Assuntos
Neoplasias Encefálicas/patologia , Epilepsia Resistente a Medicamentos/etiologia , Neoplasias Neuroepiteliomatosas/patologia , Encéfalo/patologia , Encéfalo/fisiopatologia , Neoplasias Encefálicas/complicações , Criança , Humanos , Masculino , Neoplasias Neuroepiteliomatosas/complicações
11.
J Neurosurg ; 128(4): 1173-1177, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-28598277

RESUMO

Surgical treatment of the insula is notorious for its high probability of motor complications, particularly when resecting the superoposterior part. Ischemic damage to the pyramidal tract in the corona radiata has been regarded as the cause of these complications, resulting from occlusion of the perforating arteries to the pyramidal tract through the insular cortex. The authors describe a strategy in which a small piece of gray matter is spared at the bottom of the periinsular sulcus, where the perforating arteries pass en route to the pyramidal tract, in order to avoid these complications. This method was successfully applied in 3 patients harboring focal cortical dysplasia in the posterior insula and frontoparietal operculum surrounding the periinsular sulcus. None of the patients developed permanent postoperative motor deficits, and seizure control was achieved in all 3 cases. The method described in this paper can be adopted for functional preservation of the pyramidal tract in the corona radiata when resecting epileptogenic pathologies involving insular and opercular regions.


Assuntos
Isquemia Encefálica/etiologia , Isquemia Encefálica/prevenção & controle , Córtex Cerebral/cirurgia , Epilepsia Resistente a Medicamentos/cirurgia , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/prevenção & controle , Tratos Piramidais/lesões , Adolescente , Pré-Escolar , Eletroencefalografia , Feminino , Substância Cinzenta/cirurgia , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Tomografia Computadorizada de Emissão de Fóton Único
12.
Am J Pathol ; 187(5): 1177-1185, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28427592

RESUMO

The activation of phosphatidylinositol 3-kinase-AKTs-mammalian target of rapamycin cell signaling pathway leads to cell overgrowth and abnormal migration and results in various types of cortical malformations, such as hemimegalencephaly (HME), focal cortical dysplasia, and tuberous sclerosis complex. However, the pathomechanism underlying abnormal cell migration remains unknown. With the use of fetal mouse brain, we performed causative gene analysis of the resected brain tissues from a patient with HME and investigated the pathogenesis. We obtained a novel somatic mutation of the MTOR gene, having approximately 11% and 7% mutation frequency in the resected brain tissues. Moreover, we revealed that the MTOR mutation resulted in hyperphosphorylation of its downstream molecules, S6 and 4E-binding protein 1, and delayed cell migration on the radial glial fiber and did not affect other cells. We suspect cell-autonomous migration arrest on the radial glial foot by the active MTOR mutation and offer potential explanations for why this may lead to cortical malformations such as HME.


Assuntos
Epilepsia Resistente a Medicamentos/genética , Hemimegalencefalia/genética , Malformações do Desenvolvimento Cortical do Grupo II/genética , Serina-Treonina Quinases TOR/genética , Animais , Células Cultivadas , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Feminino , Hemimegalencefalia/cirurgia , Humanos , Lactente , Malformações do Desenvolvimento Cortical do Grupo II/cirurgia , Camundongos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Serina-Treonina Quinases TOR/metabolismo , Transfecção , Regulação para Cima
13.
Springerplus ; 5(1): 1434, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27652010

RESUMO

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. At follow-up, the patient's neurodevelopmental status has been improved since the surgery. DISCUSSION: It is generally accepted that early-onset seizures in children with SWS are associated with worse neurological and developmental outcomes. However, when surgical treatment should be considered and how it should be performed remain a longstanding controversy. We promote early surgery in children with SWS and early-onset epilepsy. CONCLUSION: We suggest that VPH may be a useful adjuvant in the management of SWS with refractory epilepsy in early infancy and this procedure carries low neurological risk.

14.
J Neuropathol Exp Neurol ; 73(8): 798-806, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25003238

RESUMO

Epilepsy is one of the major neurologic diseases, and astrocytes play important roles in epileptogenesis. To investigate possible roles of astrocyte-related receptors in patients with intractable epilepsy associated with focal cortical dysplasia (FCD) and other conditions, we examined resected epileptic foci from 31 patients, including 23 with FCD type I, IIa, or IIb, 5 with tuberous sclerosis complex, and 3 with low-grade astrocytoma. Control samples were from 21 autopsied brains of patients without epilepsy or neurologic deficits and 5 patients with pathologic gliosis without epilepsy. Immunohistochemical and immunoblot analyses with antibodies against purinergic receptor subtypes P2RY1, P2RY2, P2RY4, potassium channels Kv4.2 and Kir4.1, and metabotropic receptor subtypes mGluR1 and mGluR5 were performed. Anti-glial fibrillary acidic protein, anti-NeuN, and anti-CD68 immunostaining was used to identify astrocytes, neurons, and microglia, respectively. Most glial fibrillary acidic protein-immunopositive astrocyte cells in the brain samples from patients with epilepsy were P2RY1-, P2RY2-, P2RY4-, Kv4.2-, Kir4.1-, mGluR1-, and mGluR5-positive, whereas samples from controls and pathologic gliosis showed lower expression levels of these astrocyte-related receptors. Our findings suggest that, although these receptors are necessary for astrocyte transmission, formation of the neuron-glia network, and other physiologic functions, overexpression in the brains of patients with intractable epilepsy may be associated with activation of intracellular and glio-neuronal signaling pathways that contribute to epileptogenesis.


Assuntos
Encéfalo/patologia , Diplopia/complicações , Diplopia/patologia , Epilepsia/complicações , Epilepsia/patologia , Neuroglia/metabolismo , Adolescente , Adulto , Encéfalo/metabolismo , Contagem de Células , Criança , Pré-Escolar , Feminino , Proteína Glial Fibrilar Ácida/metabolismo , Humanos , Lactente , Masculino , Canais de Potássio Corretores do Fluxo de Internalização/metabolismo , Receptores de Glutamato Metabotrópico/metabolismo , Receptores Purinérgicos P2X/genética , Receptores Purinérgicos P2X/metabolismo , Adulto Jovem
15.
J Child Neurol ; 29(3): 415-20, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23266943

RESUMO

A male infant with trisomy 21 simultaneously showed clinical features of hypomelanosis of Ito and hemimegalencephaly, with related intractable epileptic seizures. The epileptic seizures were refractory to conventional antiepileptic drugs and persisted until the patient underwent functional hemispherotomy. It is well known that patients with hypomelanosis of Ito may also have cortical dysplasia and hemimegalencephaly and that approximately half of these patients have chromosomal abnormalities. However, to our knowledge, there is no previous report of a patient with trisomy 21 associated with hemimegalencephaly. Here, we describe a rare case of coexisting trisomy 21 and hypomelanosis of Ito, associated with hemimegalencephaly.


Assuntos
Síndrome de Down/complicações , Hipopigmentação/complicações , Malformações do Desenvolvimento Cortical/complicações , Abdome/patologia , Encéfalo/patologia , Encéfalo/fisiopatologia , Encéfalo/cirurgia , Síndrome de Down/patologia , Síndrome de Down/fisiopatologia , Eletroencefalografia , Humanos , Hipopigmentação/patologia , Lactente , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/patologia , Malformações do Desenvolvimento Cortical/fisiopatologia , Malformações do Desenvolvimento Cortical/cirurgia , Convulsões/etiologia , Convulsões/patologia , Convulsões/fisiopatologia , Convulsões/cirurgia , Tórax/patologia
16.
Brain Dev ; 36(1): 74-9, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24370003

RESUMO

An 8-year-old boy underwent a resection for focal cortical dysplasia at the left supplementary motor area (SMA) for the treatment of intractable epilepsy. The manifestations of SMA syndrome, such as transient mutism and right hemiparesis, resolved within a few weeks. Verbal disfluency and impaired executive function, accompanied by impulsivity and distractibility, persisted for more than 12months. The verbal and behavioral problems caused serious difficulties in the school life of the patient, until they became less evident at 18months after surgery. Tractography performed 18months after surgery revealed a defect in the subportion of fronto-parietal association fibers within the left superior longitudinal fascicles. Verbal influency can persist with unusually long duration after resection of SMA during childhood. Although not discernible on the routine neuroimaging, white matter damage beneath the SMA region could result in serious disabilities in executive function. These complications should be recognized for the prediction and assessment of deficits in children after surgical intervention involving this region.


Assuntos
Transtornos da Linguagem/etiologia , Malformações do Desenvolvimento Cortical/cirurgia , Transtornos Mentais/etiologia , Córtex Motor/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/fisiopatologia , Criança , Humanos , Imageamento por Ressonância Magnética , Masculino , Comportamento Verbal/fisiologia
17.
Epilepsy Behav ; 29(1): 30-5, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23933627

RESUMO

This study aimed to identify the effect of early hemispherotomy on development in a consecutive series of 12 infants with hemimegalencephaly (HME) demonstrating epileptic encephalopathy. Mean age at onset was 20.4 days (range, 1-140), mean age at surgery was 4.3 months (range, 2-9), and mean follow-up time was 78.8 months (range, 36-121). Eleven patients had a history of early infantile epileptic encephalopathy. Vertical parasagittal hemispherotomy was performed without mortality or severe morbidities. At follow-up, seizure freedom was obtained in 8 patients (66.7%), who showed significantly higher postoperative developmental quotient (DQ) (mean, 31.3; range, 7-61) than those with seizures (mean, 5.5; range, 3-8) (p=0.02). Within the seizure-free group, postoperative DQ correlated with preoperative seizure duration (r=-0.811, p=0.01). Our results showed that shorter seizure duration during early infancy could provide better postoperative DQ in infants with HME and epileptic encephalopathy.


Assuntos
Desenvolvimento Infantil/fisiologia , Hemisferectomia/métodos , Malformações do Desenvolvimento Cortical/etiologia , Malformações do Desenvolvimento Cortical/cirurgia , Espasmos Infantis/complicações , Resultado do Tratamento , Eletroencefalografia , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino , Neuroimagem , Avaliação de Resultados em Cuidados de Saúde , Estatísticas não Paramétricas
18.
Brain Dev ; 35(8): 802-9, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23694756

RESUMO

PURPOSE: To describe operative procedures, seizure control and complications of surgery for cortical dysplasia (CD) causing intractable epilepsy in infancy and early childhood. METHODS: Fifty-six consecutive children (less than 6years old) underwent resective epilepsy surgery for CD from December 2000 to August 2011. Age at surgery ranged from 2 to 69months (mean 23months) and the follow-up was from 1 to 11years (mean 4years 4months). RESULTS: Half of the children underwent surgery during infancy at an age less than 10months, and the majority (80%) of these infants needed extensive surgical procedures, such as hemispherotomy and multi-lobar disconnection. Seizure free (ILAE class 1) outcome was obtained in 66% of the cases (class 1a; 55%): 85% with focal resection (n=13), 50% with lobar resection (n=18), 71% with multilobar disconnection (n=7) and 67% with hemispherotomy (n=18). Peri-ventricular and insular structures were resected in 23% of focal and 61% of lobar resections. Repeated surgery was performed in 9 children and 5 (56%) became seizure free. Histological subtypes included hemimegalencephaly (16 patients), polymicrogyria (5 patients), and FCD type I (6 patients), type IIA (19 patients), type IIB (10 patients). Polymicrogyria had the worst seizure outcome compared to other pathologies. Surgical complications included 1 post-operative hydrocephalus, 1 chronic subdural hematoma, 2 intracranial cysts, and 1 case of meningitis. No mortality or severe morbidities occurred. CONCLUSIONS: Early surgical intervention in children with CD and intractable seizures in infancy and early childhood can yield favorable seizure outcome without mortality or severe morbidities although younger children often need extensive surgical procedures.


Assuntos
Córtex Cerebral/cirurgia , Malformações do Desenvolvimento Cortical/cirurgia , Córtex Cerebral/patologia , Pré-Escolar , Eletroencefalografia/métodos , Epilepsia/etiologia , Epilepsia/fisiopatologia , Epilepsia/cirurgia , Feminino , Humanos , Lactente , Masculino , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/fisiopatologia , Complicações Pós-Operatórias/prevenção & controle , Reoperação/efeitos adversos , Reoperação/métodos , Resultado do Tratamento
19.
Brain Dev ; 35(8): 786-92, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23489890

RESUMO

PURPOSE: We studied children younger than 6years old who developed catastrophic epilepsy and were registered in the FACE study group to clarify their clinical characteristics and prevalence of seizure as well as epilepsy types. SUBJECTS: Subjects were prospectively recruited from children with epilepsy who satisfied the following criteria and underwent intensive examination between 2009 and 2012 in 14 collaborative centers: (1) younger than 6years old and (2) more than 10 seizures/month refractory to all available medical treatments including ACTH therapy, leading to significant psychosocial morbidity. METHODS: We analyzed epilepsy onset age, predominant seizure type, etiology, neuropsychological findings, and syndromic classification according to the pre-determined registration format. RESULTS: A total of 314 children were enrolled in this study. Epilepsy onset age in 239 cases (80%) was younger than 12months. The most frequent seizure type was epileptic spasms (ES), followed by generalized tonic seizures (GTS), which accounted for 42% and 20%, respectively. West syndrome (WS) was the most frequent epileptic syndrome and accounted for 37%, followed by unclassified epilepsy at 21%, neocortical epilepsy at 19%, Lennox-Gastaut syndrome at 12%, Dravet syndrome at 4%, Rasmussen syndrome at 2%, and others. The two most frequent causes of epilepsy were cortical dysplasia and chromosomal anomalies, as shown in 16% and 6%, respectively. However, the etiology of nearly one half of all patients remained unknown. Psychomotor development was already worse than a moderate degree in 62% of subjects at the first examination. CONCLUSION: The highest proportion of catastrophic epilepsy was WS and its related syndromes featuring ES and GTS, followed by neocortical epilepsy, whose psychomotor development was significantly retarded at examinations.


Assuntos
Encéfalo/fisiopatologia , Epilepsia/fisiopatologia , Idade de Início , Pré-Escolar , Diagnóstico Diferencial , Eletroencefalografia/métodos , Epilepsia/etiologia , Feminino , Humanos , Lactente , Masculino
20.
Neurol Med Chir (Tokyo) ; 53(1): 47-52, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23358171

RESUMO

Extensive multilobar cortical dysplasias occasionally occur in children and can induce seizure onset in early infancy, causing severe epileptic encephalopathy. Surgical interventions in early infancy, such as disconnection of large parts of the brain, are challenging because of the degree of invasiveness and carry greater risks in infants compared with older children. Here we report the successful treatment of intractable epilepsy with multilobar cortical dysplasias in the posterior cortex by posterior disconnection in three infants (age 3 months). The patients showed good postoperative recovery and exhibited excellent seizure control at follow-up evaluation within a year after surgery. Developmental catch-up was also achieved and no early complications have been detected to date. Use of the posterior disconnection technique for early-stage extensive multilobar cortical dysplasias can result in good seizure control and developmental progress with little perioperative morbidity. However, the efficacy of this surgical technique needs to be verified with long-term follow up after surgery.


Assuntos
Lobectomia Temporal Anterior/métodos , Epilepsia Tônico-Clônica/cirurgia , Malformações do Desenvolvimento Cortical/cirurgia , Espasmos Infantis/cirurgia , Córtex Cerebral/patologia , Eletroencefalografia , Feminino , Seguimentos , Humanos , Interpretação de Imagem Assistida por Computador , Imageamento Tridimensional , Lactente , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/diagnóstico , Complicações Pós-Operatórias/diagnóstico , Reoperação , Espasmos Infantis/diagnóstico , Técnica de Subtração , Tomografia Computadorizada de Emissão de Fóton Único
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