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Gray matter heterotopias,which can be classified into subependymal heterotopia,subcortical heterotopia,band heterotopia and mixed heterotopia,belongs to neuronal migration disorders.Any factors including physical or chemical factors,biological factors and genetical factors that occur during neuronal migration phase can lead to gray matter heterotopias.The latest studies have revealed many genes are involved in the pathogenesis of gray matter heterotopias,especially in subependymal heterotopia and band heterotopia with their own characteristics.The main clinical disorders in pediatric heterotopias include developmental delay,epilepsy and motor disturbance.The diagnosis of the disease is made through neuroimaging examinations especially magnetic resonance imagings.The advanced magnetic techniques like magnetic resonance spectroscopy and functional magnetic resonance can be helpful in the final diagnosis.
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@#A 22-year-old woman with bilateral perisylvian schizencephaly was reported. She presented only mild right hemiplegia. The motor function improved after constraint-induced movement therapy.
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Objective To analyze the CT appearances and the classification of gray matter heterotopia.Methods The clinical and CT data of 26 cases with gray matter heterotopia were retrospectively reviewed,11 were males and 15 were females,ranged in age from 2 days to 9 years with a mean of 2.6 years.Results The classification of heterotopia included:(1)Subependymal heterotopia in 14,5 cases with encephaloceles,1 case with Dandy-Walker malformation and 1 case with arachnoid cyst of cisterna megna.(2)Subcortical heterotopia in 7,4 cases with callosal agenesis were accompanied.(3)Band heterotopia in 5.Conclusion CT scan can not only reveal the appearances of the subependymal,subcortical and band types heterotopia,but also show other associated abnormalities.
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Schizencephaly is an infrequent congenital disorder of neuronal migration characterized by gray matter-lined clefts that extent through the entire cerebral hemisphere, from the ependymal lining of the lateral ventricle to the pial covering of the cortex. We have studied seven patients with schizencephaly retrospectively to correlate clinical outcome with the type, Size, and location of the clefts and to find associated brain anomalies. Three patients had bilateral clefts(one with two open lip clefts and the other two with a left open lip cleft and a right closed lip cleft), another two patients had left unilateral open lip clefts, and the rest two patients had right closed lip clefts. Clinically, these patients presented motor dysfunction such as hemiparesis, seizures, and variable developmental delay. Patients with bilateral clefts, particularly open lip type, had worse motor and developmental impairment than those with unilateral clefts. Patient with unilateral medium open lip cleft had worse motor and inte llectual impairment than those with unilateral closed lip clefts or small open lip cleft. All patients with frontal lobe involvement had motor dysfunction. Absence of septum pellucidum, gray matter heterotopia, polymicrogyria, and hypoplasia of optic nerves were also frequently found to be associated with schizencephaly. Three patients with skull canges such as enlargement of the hemicranium or erosion of the inner table of the skull were shunted but only one patient improved in the imaging study. We conclude that the severity of patient's symptoms is related to the amount, type, and location of the involved brain.