A Golgi study of the polymicrogyric cortex in Aicardi syndrome |
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| Authors: | I Ferrer M V Cusí A Liarte J Campistol |
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| Institution: | 1. Unidad de Neuropatologia, Departamento de Anatomía Patológica, Hospital Principes de España, Hospitalet de Llobregat;2. Servicio de Anatomía Patológica, Hospital Infantil San Juan de Dios, Barcelona;3. Servicio de Neuropediatría, Hospital Infantil San Juan de Dios, Barcelona;4. Servicio de Pediatría, Hospital de Gerona, Gerona, Spain.;1. Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan;2. College of Medicine, Chang Gung University, Taoyuan, Taiwan;3. Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan;4. Department of Ophthalmology, Penang General Hospital, Malaysia;5. Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan;1. PET Center, Children''s Hospital of Michigan, Detroit Medical Center, Detroit, Michigan;2. Department of Pediatrics, Wayne State University School of Medicine, Children''s Hospital of Michigan, Detroit Medical Center, Detroit, Michigan;3. Department of Neurology, Wayne State University School of Medicine, Children''s Hospital of Michigan, Detroit Medical Center, Detroit, Michigan;4. Department of Radiology, Wayne State University School of Medicine, Children''s Hospital of Michigan, Detroit Medical Center, Detroit, Michigan;5. Division of Pediatric Neurology, Nemours Alfred I. duPont Hospital for Children, Wilmington, Delaware;6. Department of Neurology, Thomas Jefferson University School of Medicine, Philadelphia, Pennsylvania;7. Department of Pediatrics, Thomas Jefferson University School of Medicine, Philadelphia, Pennsylvania |
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| Abstract: | The neuropathological examination of the brain of a 4-month-old girl with Aicardi syndrome (infantile spasms, chorioretinal lacunae, psychomotor retardation, agenesis of the corpus callosum and vertebral anomalies) showed agenesis of the corpus callosum, agenesis of the anterior commissure and abnormal orientation of the hippocampal formation, in addition to periventricular cerebral nodules and extensive non-laminated cortical polymicrogyria of the fronto-parietal regions and gyrus cinguli of both hemispheres. A non-communicating medial supracollicular cyst, racemose cysts of the choroid plexus, underdevelopment of the inferior cerebellar vermis tonsils were also observed. The study of the polymicrogyric cortex with the Golgi method revealed an abnormal orientation of neurons; but the different varieties of cells were located at the depth corresponding to that were they are normally found. The Golgy study of the periventricular nodules disclosed the presence of different types of cortex-like neurons. On the basis of these data it may be suggested that polymicrogyria is not a migratory disturbance, but rather the result of a partial necrosis of the cortical mantle occurring before the 5th month of foetal life. |
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