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Trisomies Down syndrome (risomy 21) Brain more rounded (reduced frontal lobe) Usually decreased brain weight Narrowing of superior temporal gyrus; bilateral in half of cases Brain stem and cerebellum small vs cerebrum, UUSD/CAD ganglia calcification Loss of neurons focally (cortical layer 3), poor myelination Seizures Alzheimers disease-like changes in middle- aged patients May see pathology in brain (abscess, embolic disease) related to congenital heart abnormali- ties that can be seen in Down syndrome Patau syndrome (trisomy 13) Holoprosencephaly and arrhinencephaly two- thirds of cases May see cyclopia, microcephaly, agenesis of corpus callosum, Dandy-Walker complex, hippocampal dysplasia, and cerebellar dysplasia Edwards syndrome (trisomy 18) Microcephaly, dolichocephaly, holoprosen- cephaly, dysplasia, disorganization of the lateral geniculate nucleus, malformed ears, ponto- cerebellar hypoplasia, and agenesis of corpus callosum Trisomy 9 May also see intrauterine growth retardation, skin dysplasia, organ hypoplasia, and facial anomalies Micropolygyria (polymicrogyria) Miniature, incomplete convolution Abnormal cortical architecture (four-layer cortex, absent molecular layer), thin cortical ribbon Associated with cerebellar microgyria Associated with neuronal migration abnormali- ties between 2024 weeks gestation Zellweger syndrome Pachygyria and polymicrogyria; poor cortical layering Peroxisome abnormality Chromosomes 7 (peroxin gene), 8 (Pex2), (chorioretinopathy, mental retardation, infantile spasms) Associated with webbed toes, funnel chest, spina bifida, persistent Todat, facial asym- metry, high arched palate, agenesis of cerebellar vermis, and ventricular septal defect Abnormalities of the septum pellucidum Agenesishypoplasia Associated with agenesis of corpus callosum, holoprosencephaly, defects of lateral ventri- cles and optic nerve Associated with septo-optic dysplasia- decreased visual acuity, hypothalamic- pituitary disturbances, HEXS1 mutations in some cases (chromosome 3p21) Cavum septi pellucidi Normal in newborn, separation of the leaflets of the septum pellucidum Asymptomatic x Contents 12 MRI-Guided Thermal Therapy for Brain Tumors Ferenc A.

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