Heterotopía nodular periventricular, objetivo quirúrgico en epilepsia refractaria al manejo farmacológico / Periventricular Nodular Heterotopia, Surgical Goal in Drug-Resistant Epilepsy / Heterotopia Nodular Periventricular, objetivo cirúrgico em epilepsia refratária ao manejo farmacológico

Introducción: La heterotopía neuronal es un defecto de la migración en el cual estas células no completan su desplazamiento hacia la corteza. La forma más comúnmente reportada es la heterotopía nodular periventricular, caracterizada por conglomerados neuronales que se ubican adyacentes a las paredes de los ventrículos laterales. Hasta el 90% de los pacientes con esta condición presentan epilepsia en algún momento de la vida y una gran proporción de ellos serán refractarios al manejo farmacológico. Esto hace necesario un adecuado abordaje diagnóstico que busque establecer qué pacientes se beneficiarían de resección quirúrgica de la lesión, que en la mayoría de los casos ofrece una alta tasa de control de crisis. Desarrollo: Se presenta un recorrido desde la práctica por los aspectos con mayor relevancia en cuanto a la fisiopatología, manifestaciones clínicas, abordaje diagnóstico y terapéutico de la heterotopía nodular periventricular, con el fin de explorar el rol de esta condición como causante de epilepsia refractaria. Conclusión: La epilepsia refractaria al tratamiento condiciona de manera significativa la calidad de vida de los pacientes. Una entidad frecuentemente asociada a esto es la heterotopía nodular periventricular, la cual debe ser correctamente abordada por el equipo médico tratante procurando un diagnóstico oportuno y definiendo qué pacientes se benefician del manejo quirúrgico. De esta manera, se impacta positivamente la calidad de vida de estos sujetos y de sus cuidadores.

Introduction: Neuronal heterotopia is a migration disorder in which these cells do not complete their movement toward the cerebral cortex. Periventricular nodular heterotopia is the most frequently reported form, characterized by neuronal conglomerates adjacent to the lateral ventricles walls.About 90 % of patients with this condition suffer epilepsy at some point in their lives and the major proportion of them will be resistant to pharmacologic treatment. This makes an appropriate diagnostic approach necessary in order to determine which patients would benefit from surgical resection of the lesion, which in most cases offers a high rate of crisis control. Development: This article presents a review of the most important topics approached from the practice of periventricular nodular heterotopia pathophysiology, clinical features, diagnosis and therapy. It is aimed at exploring the role of this condition as a cause of intractable epilepsy. Conclusion: Pharmacologic treatment for resistant epilepsy will have a severe impact on patient's quality of life. Periventricular nodular heterotopia is frequently associated to this condition, which must be successfully approached by the medical team attempting to an opportune diagnosis and defining which patients would benefit from surgical management. This positively impacts the quality of life of these patients and their caregivers.

Introdução: A heterotopia neuronal é um defeito da migração no qual estas células não completam seu deslocamento ao córtex. A forma mais comunmente reportada é a heterotopia nodular periventricular, caracterizada por conglomerados neuronais que se localizam adjacentes às paredes dos ventrículos laterais. Até o 90% dos pacientes com esta condição apresentam epilepsia em algum momento da vida e uma grande proporção deles, serão refratários ao manejo farmacológico. Isto faz necessária uma adequada abordagem diagnóstica, buscando estabelecer quais pacientes se beneficiariam de ressecção cirúrgica da lesão, que na maioria dos casos oferece uma alta taxa de controle de crises. Desenvolvimento: Se apresenta um recorrido desde a prática pelos aspectos com maior relevância em quanto à fisiopatologia, manifestações clínicas, abordagem diagnóstica e terapêutica da heterotopia nodular periventricular. Com o fim de explorar o rol desta condição como causador de epilepsia refratária. Conclusão: A epilepsia refratária ao tratamento condiciona de maneira significativa à qualidade de vida dos pacientes. Uma entidade frequentemente associada a isto é a heterotopia nodular periventricular, a qual deve ser corretamente abordada pela equipe médica tratante procurando um diagnóstico oportuno e definindo quais pacientes se beneficiam de manejo cirúrgico. Desta forma impacta-se positivamente a qualidade de vida destes sujeitos e de seus cuidadores.

Trastornos de la migración neuronal: un caso de lisencefalia / Neuronal migration disorders: a lissencephaly case

Las malformaciones del desarrollo cortical originan un grupo de patologías, que cursan con diversas manifestaciones las cuales marcan un grado de minusvalía significativo en quienes las padecen. La Lisencefalia es una de las alteraciones de la migración neuronal caracterizada por una corteza cerebral de superficie lisa o con pocas circunvoluciones. Este trastorno está frecuentemente asociado a epilepsias de difícil manejo. Se presenta el caso de un paciente masculino de 18 meses de edad con diagnóstico de Lisencefalia quien cursa con convulsiones tónicas generalizadas desde los 8 meses de edad. Se le realizó TAC de cráneo simple y resonancia magnética de cerebro para confirmar el diagnóstico y se dio manejo farmacológico a las convulsiones.

Malformations of cortical development originate a group of pathologies that involve diverse manifestations that mark a significant degree of disability in those who suffer from. The Lissencephaly is one of alterations in neuronal migration characterized by a smooth surface or with few convolutions in the cerebral cortex. This disorder is often associated with difficult management Epilepsies. We report the case of a male patient from 18 months of age with a diagnosis of Lissencephaly, who presents generalized tonic seizures from 8 months of age. Was performed a simple skull CT and brain magnetic resonance to confirm the diagnosis and the pharmacological management was given to seizures.

[ case report of manic episode in a patient with neuronal migration anomaly]

Neuronal migration disorders of the brain cortex are at diverse group of brain abnormalities with different etiologies and entities. They may present with various neurological and ocular abnormalities, while psychiatric presentation is very rarely reported. Here we described the first case of mania in a patient with neuronal migration disorders. We report a 27-year-old male with symptoms of mania with a prior history of occasional seizure attacks whose MRI showed neuronal migration disorder and his symptoms was controlled with sodium valproate. Association between neuronal migration disorders and mania was shown in this case. Besides, it raises the possibility that, as shown by other researchers, there may be common basis between mood disorder and neuronal migration disorders

Bilateral Frontal Polymicrogyria: An Autopsy Case Report

Bilateral frontal polymicrogyria is a recently recognized syndrome characterized by symmetric polymicrogyria of both frontal lobes that presents with delayed motor and language development, spastic quadriparesis, and variable mental retardation. However, the postmortem findings of this syndrome are not fully elaborated. Here we describe an autopsy case of bilateral frontal polymicrogyria in a male fetus delivered at 22 weeks gestation due to extensive chorioamnionitis. The microscopic findings included a thinned cortical plate with fair neuronal maturation. There were no signs of neuronal damage and the white matter was unremarkable.

Patología de las malformaciones del desarrollo cortical en pacientes con epilepsia refractaria: experiencia en un hospital pediátrico / Pathology of CDM in patients with refractory epilepsy: experience in a pediatric hospital

Las malformaciones del desarrollo cortical (MDC) comprenden un grupo heterogéneo de lesiones caracterizadas por una desorganización de la arquitectura normal de la corteza cerebral, que incluye desde cambios microscópicos sutiles a extensas lesiones que pueden comprometer un hemisferio entero o conformar síndromes genéticos definidos. Múltiples esquemas de clasificación han sido propuestos para catalogar pacientes con malformaciones del desarrollo en subgrupos, basados en criterios de imágenes y hallazgos patológicos. Sin embargo aun hoy, la bibliografía es muy confusa en cuanto a su nomenclatura y/o a los criterios utilizados para evaluar la reproducibilidad. En algunos casos puede ser difícil distinguir estas lesiones de un tumor (ganglioglioma o Tumor Neuroepitelial Disembrioplásico) por la posible coexistencia de ambas lesiones y la representatividad de las muestras. La utilización del tejido humano de las resecciones quirúrgicas provee una fundamental herramienta tanto para esclarecer la patogénesis como para futuras investigaciones. Nuestra experiencia en patología de la epilepsia relacionada con malformaciones del desarrollo a lo largo de 20 años en un hospital público pediátrico incluye patología del lóbulo temporal y extratemporal, con lesiones puras malformativas, patología dual y otras asociaciones.

Malformations of cortical development (MCD) comprise a heterogeneousgroup of lesions characterized by a disruption of the normal architecture of the cerebral cortex, ranging from subtlemicroscopic lesions to large lesions that can compromise an entire hemisphere or shape defined genetic syndromes. Multiple classification schemes have been proposed to catalogue patients with developmental malformations in subgroups based on criteria and pathological images. But even now, literature is very confusing in their classification and / or the criteria used to assess reproducibility. In some cases it may be difficult to distinguish these lesions from a tumor (ganglioglioma orDysembryoplastic Neuroepithelial Tumor) for the possible coexistenceof both lesions and representativeness of the samples. The use of human tissue for surgical resection provides afundamental tool both to clarify the pathogenesis and for future research. Our experience in pathology of epilepsy associated with malformations of the development over 20 years in a public pediatric hospital includes temporal lobe and extratemporal pathology, pure malformations, dual pathology and other associations.

Áreas corticales elocuentes: estudio con RMF bases fisiológicas, procedimiento y aplicaciones clínicas / Eloquent cortical areas: RMF study physiological bases, procedures and clinical applications

El conocimiento de la anatomía funcional del cerebro es útil para correlacionar síntomas específicos con la localización de lesiones en áreas específicas. La RM funcional es una herramienta diagnóstica no invasiva que permite estudiar funciones cerebrales en base a una técnica denominada BOLD (blood oxygenation level dependent). Si bien la principal indicación de la RMf corresponde a la evaluación prequirúrgica de tumores cerebrales, esta metodología diagnóstica también es útil, entre otras aplicaciones, para el estudio de la lateralización del lenguaje, de la memoria y de la neuroplasticidad como mecanismo compensador de daño cerebral de origen traumático o vascular.

The knowledge of the brain functional anatomy is useful to link specific symptoms with the lesion´s location in specific areas. Functional MRI is a non-invasive diagnostic tool able to visualize brain function with a technique named BOLD(blood oxygenation level dependent). The most important indication of fMRI is for presurgical planning in patients with brain tumors, moreover this technique can use to study language lateralization, memoryand neuroplasticity, among other functions.

Seizure characteristics in Aicardi syndrome

We are describing a one year old Bahraini girl with Aicardi syndrome; she has agenesis of the corpus callosum, inter hemispheric cyst, neuronal migration disorder, ocular abnormalities and infantile spasm. She presented initially with focal seizure which later evolved into infantile spasm, her seizures were intractable to different anticonvulsant medications except for Vigabatrin which reduced her seizure episodes dramatically

Immunohistochemical study of acute effects of ethanol on midbrain of wistar rats associated with the date of birth of neurons in encephalon / Estudio inmunohistoquímico de los efectos agudos del etanol sobre el mesencéfalo de ratas wistar asociado con la fecha de formación de neuronas en el cerebro

The verification of the acute effects of ethanol on the midbrain, which neurons are mainly formed in E14, will ratify if the acute effects of ethanol are associated, in terms of prevalence, to the date of birth of neurons in certain regions of the brain. The aim of this study was to determine the effects of acute exposure to ethanol on the neuronal density of the midbrain and to associate these results with acute effects of ethanol in other regions of the brain under the same conditions. The rats were treated with intraperitoneal ethanol during one day (E12) and 1h after the last ethanol injection was applied to BrdU. The animals were sacrificed; the brains were removed and sectioned. The sections were treated with DAB for the BrdU revelation, the slides were stained with safranin for one group and another group was stained with H & E. The effects of acute injection of ethanol in E12 on the midbrain were not detected in this study. Data from literature suggest that the deleterious action of acute ethanol consumption only occur if the drug is injected at times near the beginning of the migration of neurons in that particular region of the brain.

La verificación de los efectos agudos del etanol sobre el mesencéfalo, cuyas neuronas se forman principalmente en la E14, puede ratificar si los efectos agudos del etanol están asociados, en términos de prevalencia, a la fecha de nacimiento de neuronas en ciertas regiones del cerebro. El objetivo de este estudio fue determinar los efectos de la exposición aguda al etanol sobre la densidad neuronal del mesencéfalo y asociar estos resultados con los efectos agudos de etanol en otras regiones del cerebro bajo las mismas condiciones. Las ratas fueron tratadas con etanol intraperitoneal durante un día (E12) y 1h después de la última inyección de etanol fue aplicado BrdU. Los animales fueron sacrificados, los cerebros fueron removidos y seccionados. Las secciones fueron tratados con DAB para la revelación de BrdU, las secciones fueron teñidas con safranina para un grupo y otro grupo se tiñeron con H&E. Los efectos agudos de la inyección de etanol en E12 sobre el mesencéfalo no fueron detectados en este estudio. Los datos de la literatura sugieren que los efectos deletéreos del consumo agudo de etanol sólo se producen si la droga se inyecta en etapas cercanas al comienzo de la migración de las neuronas en esa región particular del cerebro.

Displasias Corticais / Cortical Dysplasias

Este artigo revisa bases fisiopatológicas e classificação das displasias corticais. Enfocamos a importância do diagnóstico precoce, através dos métodos clássicos e novos. Além disso, abordamos as terapêuticas existentes e as situações em que devem ser utilizadas.

Effects of Ethosuximide on the Pilocarpine Induced Seizure in Rat Model of Neuronal Migration Disorder

Cortical malformation-associated epileptic seizures are resistant to conventional anticonvulsant drugs. Relatively little research has been conducted on the effects of antiepileptic drugs (AEDs) on seizure activity in a rat model of dysplasia. We have used rats exposed to methylazoxymethanol acetate (MAM) in utero, an animal model featuring nodular heterotopia, to investigate the effects of ethosuximide (ETX) in the dysplastic brain. Pilocarpine was used to induce acute seizure in MAM-exposed and age-matched vehicle-injected control animals. Field potential recordings were used to monitor the amplitude and number of population spikes, and paired pulse inhibition in response to stimulation of the commissural pathway. Pharmaco-resistance was tested by measuring seizure latencies after pilocarpine administration (320 mg/kg, i.p.) with and without pre-treatment with ETX. Pre-treatment with 300 mg of ETX significantly prolonged the latency to the status epilepticus (SE) in both control and MAM-treated groups. Pre-treatment with ETX 100mg and ETX 200 mg had little effect in MAM-exposed rats. However, ETX 200 mg prolonged the latency to the SE in control groups. Spontaneous field potential and secondary after-discharges were higher for MAM-treated rat in comparison with control rats injects with ETX. The main findings of this study are that acute seizures initiated in MAM-exposed rats are relatively resistant to standard ETX assessed in vivo. These data suggest that ETX do not prolong seizure latencies in MAM-rats exposed to pilocarpine.

A Study of Ulegyria as Pathognomonic Aspects of Congenital Bilateral Perisylvian Syndrome

OBJECTIVE: Congenital bilateral perisylvian syndrome (CBPS) has been defined as a characteristic malformative perisylvian polymicrogyria (PMG) in patients with clinical symptoms of pseudobulbar palsy and epileptic seizures. For the present study, we investigate clinicopathologic features of CBPS associated with timing of lesion formation. METHODS: Clinicopathologic features of CBPS from 6 patients with surgical resection of the cerebral lesions due to medically intractable seizures were studied. RESULTS: Seizure onset ranged from 1 to 10years (average 6.7years) of age, and average duration of seizure was 23years. All had complex partial seizures, and two patients had additional tonic clonic seizures. Magnetic resonance (MR) images showed polymicrogyria, atropic gyri with gliosis. In the histopathologic examination, the cortical lesions revealed features of ulegyria ; atrophic and sclerotic gyri, laminar loss of neurons, extensive lobular gliosis throughout the gray and white matter, neuronoglial nodule formation, and many amyloid bodies. Unlayered or four-layered PMG was not identified. CONCLUSION: Above data suggest that CBPS might be caused by ulegyria resulting from developmental cortical defect during early fetal stage or acquired hypoxic/ischemic injury in prenatal or postnatal life.

Relationship between Cavum Septum Pellucidum and Epilepsy

OBJECTIVE: The authors study a relationship between the presence of cavum septum pellucidum(CSP) and the development of epilepsy by comparing the presence of CSP, which has been known to be a normal variation, in normal control group and epilepsy patients. METHODS: This study included 377 patients with epilepsy and 252 controls without epilepsy. Of epilepsy patients, 168 patients underwent surgery due to intractability and 209 patients was on medication of antiepileptic drugs. Control group had only headache and no visible lesion in MRI. Of 168 surgical patients, 102 patients had temporal lobe epilepsy and 66 patients had extratemporal lobe epilepsy. Ninty five patients showed a neuronal migration disorder in histopathologic findings. Definition of "CSP" and "partial CSP" was followed by Pauling's classification. RESULTS: CSP was present 8.2% of epilepsy patients and 1.6% of control group(p<0.01). CSP was detected in 11.3% of patients with surgical treatment and in 5.7% of patients with medical treatment. CSP was noticed in 8.9% of temporal lobe epilepsy, in 15.2% of extratemporal lobe epilepsy, in 13.7% of patients with neuronal migration disorder, and in 8.2% of patients with no neuronal migration disorder. CONCLUSION: Presence of CSP is statistically higher in epilepsy patients than in control group. This results indicates that the presence of CSP may not be a simple normal variation, and it can be considered a developmental anomaly that may contribute to epileptogenesis.

Neurofilament Protein Subtype Expression in Neuronal Migration Disorders

BACKGROUND: Neuronal migration disorder (NMD) is one of the causes of medically intractable epilepsy. As neurosurgical treatments for medically intractable epilepsy have expanded recently, precise histopathologic diagnosis is required. Histopathologic grading of NMD is important due to its association with neocortical development and expectation of prognosis. Many studies revealed abnormalities of neuronal cytoskeletal protein in abnormal neuronal cells of NMD. METHODS: We performed immunohistochemical staining for neurofilament protein (NF) subtypes, one of the neuronal cytoskeletal proteins, and investigated the staining pattern of specific cells in each grade of NMD. RESULTS: NF-L was more intensely labeled in perikarya, dendrites, and axons of normal or small sized dysplastic neurons, cytomegalic neurons, and balloon cells than of normal-looking neurons. Furthermore, positive reaction was more intense in high-grade lesion. NF-H and NF-M were mainly positive in the axons of gray and white matter and weakly positive in a few cytomegalic neurons and some balloon cells. CONCLUSION: NF-L is a better marker than NF-H and NF-M for the detection of normal or small sized dysplastic neurons, cytomegalic neurons, and balloon cells and for grading of NMD.

The Estimation of the Probable Cause and the Time of Cerebral Insult in Cerebral Palsy

OBJECTIVE: To estimate the probable cause and the time of cerebral insult in cerebral palsy (CP) based on MRI findings and risk factors. METHOD: The subjects comprised all sixty-seven patients with CP showing abnormal MRI findings between March 1999 and September 2001 at the Catholic University of Korea, St. Mary's Hospital. A detailed medical history was available for all patients including those not born in our hospital. They ranged in age from two months to five years. We analyzed the brain magnetic resonance (MR) findings of patients with CP to correlate the probable cause and the time of cerebral insult through the consideration of medical histories including prenatal, perinatal and postnatal histories. RESULTS: Of the 67 MRIs, abnormalities were the followings; periventricular leukomalacias (PVLs) in 49 cases, cortical or subcortical infarction in 4 cases, brain atrophy in 7 cases, neuronal migration disorder in 4 cases, and delayed myelination in 3 cases. Among the patients with PVL, perinatal risk factors were responsible for cerebral insult in preterm, but pre- and perinatal contribution were similar in patients born at full term. Among the patients with cerebral infarction, only one case with meningitis at 11 months was suspected for cerebral insult. These patients had no risk factor as a peri- or post-natal etiology. Four patients with neuronal migration disorder had no risk factor for peri- or postnatal etiology except for the one who was a twin. CONCLUSION: Review of brain MRI findings such as PVL, infarct, neuronal migration disorder and a detailed medical history including prenatal and perinatal etiology would be a useful method to estimate the probable cause and the time of cerebral insult in CP.

Kinetics of GABAa Receptors in An Animal Model of Irradiation-induced Neuronal Migration Disorders

PURPOSE: A majority of patients with neuronal migration disorders(NMDs) in cortical structures suffer from medically intractable epilepsy. The role of NMDs on seizure susceptibility or epileptogenecity has not been well documented. In the present study, we established an experimental model of NMDs in Sprague-Dawley rats by exposing fetal rats to external irradiation in order to demonstrate epileptogenic effect of NMDs lesions. METHODS: Pregnant rats were exposed to 240cGy of external X-irradiation delivered by a linear accelerator source on gestational day 16 and 17 to produce NMDs lesions in rat brain. RESULTS: Microcephaly was evident on gross examination of the affected brains. Seizure susceptibility was tested by a small dose of kainate(0.1mg/kg) injected intraperitoneally, and the irradiated animals showed increased susceptibility to kainate. Histopathologic examination revealed cortical dysplasia consisting of dyslamination of cerebral cortex and appearance of cytomegalic neurons, neuronal heterotopia in periventricular white matter, dispersion of pyramidal layer and hippocampal dentate gyrus, and agenesis of corpus callosum. Histopathologic change of the cerebral cortex and hippocampus was closely correlated with seizure activity. Quantitative autoradiography of [H]muscimol binding to GABAA receptors was significantly reduced in NMDs lesions(P=0.02). CONCLUSION: In utero irradiation of fetal rats resulted in histopathologic abnormalities that mi- miced several characteristic features of human neuronal migration disorders, and hyperexcitability appears to be associated with reduction in density of GABAp receptors in the brain, particularly in hippocampus and neocortex.

The Clinical Manifestations and Results of Medical and Surgical Treatment of Epileptic Children with Neuronal Migration Disorders / 대한소아신경학회지

PURPOSE: The objective of this study is to investige the clinical features, natural histories, and results of medical and surgical treatment of NMD in patients who were diagnosed during childhood. METHODS: We performed a retrospective analysis of medical records of 57 patients with NMD who were newly diagnosed by MRI or pathologically in epilepsy children since March 1993 to June 2000. RESULTS: These 57 patients with NMD consisted of 26 with cortical dysplasia, 9 with lissencephaly, 7 with polymicrogyria, 6 with schizencephaly, 4 with hemimegalencephaly, 3 with heterotopias, and 2 with double cortex. Clinically, 94.7% of these patients showed seizures, 33.3% with developmental delay, 21.1% mental retardation, 15.8% cerebral palsy, and 7.0% attention deficit hyperactivity disorder. Their response to antiepileptic drugs was good to 31 patients (75.6%), moderate to 3 (7.3%), and poor to 7 (17.1%). Twelve patients were completely seizure-free after receiving medication for at least 15 months. Seventeen patients tolerated with monotherapy with antiepileptic drugs. Fourteen patients underwent surgical resection. The results of operation were highly correlated with the complete removal of epileptic focus. Six patients who underwent complete resection were seizure-free after operation. On the other hand, Eight patients who had incomplete resection of the epileptic focus showed poor outcome. CONCLUSION: Most of previous reports suggested that NMD is associated with refractory to medical treatments, and early surgical operation has been recommended. Our study demonstrates remarkably good responses of NMD patients with medical treatment only.

Surgery of Intractable Epilepsy Associated with Cortical Dysplasia

Cortical dysplasia(CD) is recently known as a cause of intractable partial epilepsies that are amenable to surgical treatment. The development of new neuroimaging has facilitated the recognition of these neuronal migration disorders. Here we examine some clinical features that permit early suspicion of focal cortical dysplasia and better surgical results. From a consecutive surgical series of 239 patients with intractable epilepsy since 1992, pathologically verified 31 CD including 6 CD with dysembryoplastic neuroepithelial tumor(DNT) were selected for this study. The location and extent of resection were determined by both epileptogenic zones and the structural lesion, according to presurgical evaluation(neuroimaging, EEG, intracranial recording), intraoperative electrocorticography(ECoG) and functional brain mapping. The series consisted of 21 men and 10 women with ages at seizure onset ranging from 1 to 26 years(mean 11.1year). The duration of the epilepsy prior to surgery ranged from 3 to 30 years(mean 14.3). The CD was verified in 17(11.1%) of 153 cases with temporal lobe epilepsy and 8(16.6%) of 48 cases with extratemporal epilepsy, mainly peri-Rolandic area. The lesion location of CD with DNT were temporal(4 cases) and extratemporal(2 cases). The histology of the surgical specimens showed cortical dyslamination in 26 patients, additional dysplastic neurons in 2 patients, and additional balloon cells in 3 patients. Excellent and good clinical results were achieved in 29 cases. CD should be suspected when intractable partial epilepsy occur in children. Careful investigation of neuroimaging techniques with high resolution MRI and sophisticated presurgical and intraoperative tailoring is essential for better outcome with identification of CD.

Brain Magnetic Resonance Imaging Findings in Children withCerebral Palsy: Compared between Preterm and Term Groups / 대한소아신경학회지

PURPOSE: We used MRI to retrospectively analyze the brain of patients suffering from cerebral palsy. Our aim is to determine MRI's role in the assessment of brain damage, the relationship of gestational age. METHODS: A total of 66 patients(29 preform group and 37 term group), who visited Kang-Dong Sacred Heart Hospital from January, 1994 to July, 1998, were enrolled in this study. RESULTS: Among the 29 in the preform group, 13 patients showed MR images of hypoxic ischemic injury in which periventricular leukomalacid(PVL) and multifocal ischemic necrosis in 12(41.3%) and 1(3.4%) respectively. Neuronal migration disorders were 6(20.8%), other congenital malformations 5(17%) and normal MR images 5(17%) in this preform group. Among the 37 in the term group, 22 patients showed MR images of hypoxic ischemic injury in which selective neuronal necrosis were 11(29.7%), PVL 4(10.8%), focal and multifocal ischemic necrosis 4(10.8%) and status marmoratus 3(8.1%). Neuronal migration disorders were 4(10.8%), other congenital malformations 5(13.5%) and normal MR images 6(16.2%) in the term group. CONCLUSION: MRI provided useful information in a majority of children with cerebral palsy. Hypoxic ischemic injury was significantly different in preform and term groups. PVL was frequent in the preterm group and selective neuronal necrosis was statistically common in the term group.

Brain Magnetic Resonance Imaging Findings in Children with Cerebral Palsy: Compared between Preterm and Term Groups / 대한소아신경학회지

PURPOSE: We used MRI to retrospectively analyze the brain of patients suffering from cerebral palsy. Our aim is to determine MRI's role in the assessment of brain damage, the relationship of gestational age. METHODS: A total of 66 patients (29 preterm group and 37 term group), who visited Kang-Dong Sacred Heart Hospital from January, 1994 to July, 1998, were enrolled in this study. RESULTS: Among the 29 in the preterm group, 13 patients showed MR images of hypoxic ischemic injury in which periventricular leukomalacia (PVL) and multifocal ischemic necrosis in 12 (41.3%) and 1 (3.4%) respectively. Neuronal migration disorders were 6 (20.8%), other congenital malformations 5 (17%) and normal MR images 5 (17%) in this preterm group. Among the 37 in the term group, 22 patients showed MR images of hypoxic ischemic injury in which selective neuronal necrosis were 11 (29.7%), PVL 4 (10.8%), focal and multifocal ischemic necrosis 4 (10.8%) and status marmoratus 3 (8.1%). Neuronal migration disorders were 4 (10.8%), other congenital malformations 5 (13.5%) and normal MR images 6 (16.2%) in the term group. CONCLUSION: MRI provided useful information in a majority of children with cerebral palsy. Hypoxic ischemic injury was significantly different in preterm and term groups. PVL was frequent in the preterm group and selective neuronal necrosis was statistically common in the term group.

A Case of Band Heterotopia with Lennox-Gastaut Syndrome / 대한소아신경학회지

Band heterotopia is a rare neuronal migration disorder, resulting in epilepsy and mental retardation. Epilepsy in band heteropopia, of which Lennox-Gastaut syndrome constituted about 20%, varied in nature and degree of severity. Band heterotopia can be diagnosed by brain magnetic resonance imaging (MRI), showing another diffuse layer of gray matter underlying the normal-looking cortex with intervening thin rim of white matter. While positron emission tomography (PET) with [18F]-fluorodeoxyglucose revealed glucose uptake similar to the overlying cortex, single photon emission computerized tomography (SPECT) findings of band heterotopia have not been reported. We report a 8-year-old girl who presented with variable types of generalized seizures and mild mental retardation. She was diagnosed as having band heterotopia with Lennox-Gastaut syndrome by MRI and interictal electroencephalogram (EEG) showing immature background and generalized 2 Hz slow spike and wave complexes. Interictal SPECT, using Tc 99m hexamethyl propylenamine oxime (Tc 99m-HMPAO), revealed the same degree of perfusion in both the areas of band heterotopia and the overlying cortex. By using valproate and lamotrigine, she is now in stable condition with a significant decrease in seizure frequency.