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.
Amyloid ; Epilepsy ; Gliosis ; Humans ; Malformations of Cortical Development ; Neuronal Migration Disorders ; Neurons ; Pseudobulbar Palsy ; Seizures

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.
Anticonvulsants ; Classification ; Epilepsy* ; Epilepsy, Temporal Lobe ; Headache ; Humans ; Magnetic Resonance Imaging ; Neuronal Migration Disorders ; Septum Pellucidum*

Neuronal migrational disorders of the brain represent abnormalities in the formation of the neocortex caused by faulty migration of the subependymal neuroblasts. The neuroblasts normally migrate between the sixth and 15th gestational week and in doing so form the six-layered neocortex. When the migration does not occur in a normal fashion the resultant brain anomalies include lissencephaly, pachygyria, schizencephaly, hemimegalencephaly, heterotopia, and polymicrogyria. Neuronal heterotopia is a collection of nerve cells in abnormal locations as a result of arrest of their radial migration, improper formation, or destruction of the radial glial fiber. We reported a case of neuronal heterotopia with brief review of related literatures.
Agenesis of Corpus Callosum ; Brain ; Lissencephaly ; Malformations of Cortical Development ; Neocortex ; Neuronal Migration Disorders ; Neurons*

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.
Axons ; Cerebral Cortex ; Cytoskeletal Proteins ; Dendrites ; Diagnosis ; Epilepsy ; Nervous System Malformations ; Neurofilament Proteins ; Neuronal Migration Disorders* ; Neurons* ; Prognosis

Neuronal migration disorders (NMD's) are a rare group of developmental structural lesions characterized by disorganization of cortical architecture with aberrant columnar and laminar arrangement, often causing intractable seizures. During September 1994 to February 1995, we operated on six patients with NMD to treat intractable seizures, Male female ratio of these 6 patients was 2:4 and mean age at seizure onset was 14 years old(range 6-28 years), indicating early onset of epilepsy. Mean age at seizure surgery was 29 years old(range 23-41 years), and mean follow-up duration after operation was 4 months(range 3-6 months). In their past medical history, three patients had experienced febrile seizure at pediatric age, and one of them had a history of anoxic damage during delivery. Following preoperative localization, the lesion was removed completely in five patients. In the other one patient part of the lesion was located the speech and motor area, leading to partial removal. On histologic examination, two of them showed cortical dysplasia and the other four revealed microdysgenesis. During follow-up for six months, five patients were free of seizure and in the other one patient, whose lesion was removed incompletely, the frequency of seizure decreased by 95%.
Epilepsy* ; Female ; Follow-Up Studies ; Humans ; Male ; Malformations of Cortical Development ; Neuronal Migration Disorders* ; Neurons* ; Seizures ; Seizures, Febrile

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.
Animals ; Anticonvulsants ; Brain ; Epilepsy ; Ethosuximide* ; Methylazoxymethanol Acetate ; Models, Animal* ; Neuronal Migration Disorders* ; Neurons* ; Pilocarpine* ; Rats* ; Seizures* ; Status Epilepticus

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.
Brain* ; Cerebral Palsy* ; Child* ; Gestational Age ; Heart ; Humans ; Infant, Newborn ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging* ; Movement Disorders ; Necrosis ; Neuronal Migration Disorders ; Neurons ; Retrospective Studies

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* ; Cerebral Palsy ; Child* ; Gestational Age ; Heart ; Humans ; Magnetic Resonance Imaging* ; Movement Disorders ; Necrosis ; Neuronal Migration Disorders ; Neurons ; Paralysis* ; Retrospective Studies

Neuronal migration disorder is a rare group of congenital malfomations of the brain caused by insults to migrating neuroblasts during the six to fifteen gestational weeks. We identified 36 neuronal migration disorders on CTs in two patients and on MRIs in 34 patients and analyzed their characteristic radiologic, clinical, and EEG findings. These 36 patients with neuronal migration disorders consisted of 18 with schizencephaly, eight with pachygyria, five with heterotopias, three with lissencephaly, and two with polymicrogyria. Patient ranged in age from 6 months to 37 years old and mean age was 18.2 years old. Associated cerebral anomalies included ventricular dilatation in 13 patients, agenesis of septum pallucidum and hypoplasia of corpus callosum in nine patients. Lissencephaly was associated with other cerebral anomalies most frequently and all of them had ventricular dilatation and hypoplasia of corpus callosum. Only one patient with pachygyria had ventricular dilatation. Clinically, these patients presented with seizures in 91.7%, speech impairment in 33.3%, abnormal motor function in 30.5%, developmental delay in 27.8%, mental retardation in 25%. Patients with large or medium size of neuronal migration disorders had significantly more severe developmental delay(p=0.001), mental retardation (p=0.004) and speech impairment (p=0.01) than those with small size. Abnormal motor dysfunctions were not significantly associated with lesion size statistically. Seizures did not correlate with lesion size.
Adult ; Brain ; Corpus Callosum ; Dilatation ; Electroencephalography ; Humans ; Intellectual Disability ; Lissencephaly ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Neuronal Migration Disorders* ; Neurons* ; Seizures

PURPOSE: To evaluate characteristic MR findings of craniocerebral anomaly and its relationship with neurologic manifestations. MATERIALS AND METHODS: We retrospectively reviewed MR images of 36 patients with craniocerebral anomaly diagnosed by MRI and clinical courses. We correlated the characteristic MR findings in 41 lesions with neurologic manifestastions focusing on seizures and developmental delay. RESULTS: Twenty-three patients with seizures consisted of 14 patients(60%) with neuronal migration disorders and seven(30%) with phakomatosis, among which 18 patients(78%) had generalized type of seizures. Locations of the lesions were the parietal lobes in 11 patients(52%) and the subependymal or periventricular regions in seven(33%). Two patients with tuberous sclerosis had the lesions in both parietal and subependymal areas. Nine patients had the signs of developmental delay that were seen in the four(44%) with schizencephaly, two (22%) with tuberous sclerosis, two(22%) with heterotopia, and one(ll %) with pachygyria. CONCLUSION: Neuronal migration anomaly was relatively common lesion that presented neurologic manifestations such as seizures and developmental delay. Generalized type of seizures was common. We were able to diagnose these anomalies using the MRI that helped establish therapeutic plans.
Humans ; Lissencephaly ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Neurocutaneous Syndromes ; Neurologic Manifestations ; Neuronal Migration Disorders ; Neurons ; Parietal Lobe ; Rabeprazole ; Retrospective Studies ; Seizures* ; Tuberous Sclerosis