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Chapter 16 Defects of Neural Tube Closure Anencephaly Encephalocele Meningocele Myelomeningocele Structural Malformations Malformations of the Axial Skeleton Spina bifida occulta Vertebral defect that allows the protrusion of the neural tube contents Inherited Metabolic Disorders of the Central Nervous System Defects in amino acid metabolism Phenylketonuria (PKU) → hyperphenylalaninemia Defects in lipid metabolism (lysosomal storage) Tay-Sachs disease AR, -↓ hexosaminidase A, 80% Jewish Phenylketonuria Acute Encephalopathies Reye syndrome Drug induced (Table 16-6) Lead poisoning Acute Encephalopathies Meningitis Bacterial Viral HIV and CNS Involvement HIV infections in children Perinatally through the placenta Exposure to infected maternal blood and vaginal secretions Postpartum ingestion of breast milk