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Alterations of Neurologic Function in Children Chapter 19 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.

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Presentation on theme: "Alterations of Neurologic Function in Children Chapter 19 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc."— Presentation transcript:

1 Alterations of Neurologic Function in Children Chapter 19 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc.

2 2 Embryonic Development of the Neural System Dorsal (posterior) induction Dorsal (posterior) induction Ventral (anterior) induction Ventral (anterior) induction Proliferation Proliferation Migration Migration Organization Organization Myelination Myelination

3 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 3 Structure and Function of the Nervous System in Children Develops from a dorsal thickening of the ectoderm (neural plate) Develops from a dorsal thickening of the ectoderm (neural plate) Neural groove and folds Neural groove and folds Neural tube Neural tube Neural crest Neural crest Mesoderm Mesoderm Blood vessels, microglial cells, dural and arachnoid layers of the meninges, the capsule of some peripheral nerve endings, and nerve coverings Blood vessels, microglial cells, dural and arachnoid layers of the meninges, the capsule of some peripheral nerve endings, and nerve coverings

4 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 4 Structure and Function of the Nervous System in Children Sulcus limitans Sulcus limitans Basal plate Basal plate Alar plate Alar plate Sutures Sutures Fontanels Fontanels Myelin sheath Myelin sheath A lipid-protein sheath A lipid-protein sheath

5 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 5 Structure and Function of the Nervous System in Children

6 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 6 Structure and Function of the Nervous System in Children

7 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 7 Neural Tube Defects (NTD) Caused by an arrest of the normal development of the brain and spinal cord Caused by an arrest of the normal development of the brain and spinal cord Occurs in about 3000 U.S. pregnancies/yr Occurs in about 3000 U.S. pregnancies/yr Strong association of fetal death Strong association of fetal death Reduces the actual prevalence of neural defects at birth Reduces the actual prevalence of neural defects at birth Maternal folate deficiency Maternal folate deficiency Periconceptional supplementation with folic acid can reduce NTD by up to 70% Periconceptional supplementation with folic acid can reduce NTD by up to 70%

8 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 8 Structural Malformations Defects of neural tube closure Defects of neural tube closure Anencephaly Anencephaly Encephalocele Encephalocele Meningocele Meningocele Myelomeningocele Myelomeningocele Arnold-Chiari type II malformation Arnold-Chiari type II malformation Tethered cord syndrome Tethered cord syndrome

9 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 9 Structural Malformations

10 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 10 Axial Skeleton Malformations Spina bifida occulta Spina bifida occulta Vertebral defect that allows the protrusion of the neural tube contents Vertebral defect that allows the protrusion of the neural tube contents Cranial deformities Cranial deformities Acrania Acrania Craniosynostosis Craniosynostosis Microcephaly Microcephaly Congenital hydrocephalus Congenital hydrocephalus Macewen sign (cracked pot sign) Macewen sign (cracked pot sign)

11 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 11 Encephalopathies Static encephalopathies Static encephalopathies Cerebral palsy: a diverse group of nonprogressive syndromes that affect the brain and cause motor dysfunction beginning in early infancy Cerebral palsy: a diverse group of nonprogressive syndromes that affect the brain and cause motor dysfunction beginning in early infancy Static cerebral palsy Static cerebral palsy Dyskinetic cerebral palsy Dyskinetic cerebral palsy Ataxic cerebral palsy Ataxic cerebral palsy

12 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 12 Inherited Metabolic Disorders of the Central Nervous System Defects in amino acid metabolism Defects in amino acid metabolism Phenylketonuria (PKU) Phenylketonuria (PKU) Hyperphenylalaninemia Hyperphenylalaninemia Defects in lipid metabolism Defects in lipid metabolism Lysosomal storage diseases Lysosomal storage diseases Tay-Sachs disease Tay-Sachs disease

13 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 13 Seizure Disorders Epilepsy Epilepsy Partial seizures Partial seizures Generalized seizures Generalized seizures Unclassified epileptic seizures Unclassified epileptic seizures Infantile spasms Infantile spasms Lennox-Gastaut syndrome Lennox-Gastaut syndrome Juvenile myoclonic epilepsy Juvenile myoclonic epilepsy

14 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 14 Benign Febrile Seizures Occur in 2% to 5% of children Occur in 2% to 5% of children Brief and self-limited Brief and self-limited Often between ages 6 months and 5 years Often between ages 6 months and 5 years Peak incidence at 14 to 18 months Peak incidence at 14 to 18 months Pathogenesis unknown Pathogenesis unknown Influencing factors: age, degree and rate of temperature elevation, nature of illness Influencing factors: age, degree and rate of temperature elevation, nature of illness

15 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 15 Status Epilepticus Continuing or recurring seizure activity Continuing or recurring seizure activity Recovery from seizure activity is incomplete Recovery from seizure activity is incomplete Seizure activity is unrelenting Seizure activity is unrelenting Usually lasts for 30+ minutes Usually lasts for 30+ minutes Any seizure activity can evolve into status epilepticus Any seizure activity can evolve into status epilepticus Status epilepticus is a medical emergency that requires immediate intervention Status epilepticus is a medical emergency that requires immediate intervention

16 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 16 Acute Encephalopathies Reye syndrome Reye syndrome Usually associated with influenza B or varicella virus infections in children who have taken aspirin Usually associated with influenza B or varicella virus infections in children who have taken aspirin Pathology unknown Pathology unknown Inborn errors of metabolism are a contributing factor Inborn errors of metabolism are a contributing factor Profound hypoglycemia, hypoketonemia, hyperammonemia, increase in serum short-chain fatty acids Profound hypoglycemia, hypoketonemia, hyperammonemia, increase in serum short-chain fatty acids Liver shows diffuse deposits of lipids and absence of any inflammatory reaction or necrosis Liver shows diffuse deposits of lipids and absence of any inflammatory reaction or necrosis Fatty degeneration of the kidneys leads to azotemia (excess urea in the blood) Fatty degeneration of the kidneys leads to azotemia (excess urea in the blood) Brain is extremely edematous Brain is extremely edematous

17 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 17 Acute Encephalopathies Meningitis Meningitis Inflammation of the meningeal coverings of the brain and spinal cord Inflammation of the meningeal coverings of the brain and spinal cord Bacterial meningitis Bacterial meningitis 6000 cases per year; half in children younger than 18 6000 cases per year; half in children younger than 18 Viral meningitis Viral meningitis Hallmark of viral meningitis or aseptic meningitis is a mononuclear response in the CSF and the presence of normal blood glucose level Hallmark of viral meningitis or aseptic meningitis is a mononuclear response in the CSF and the presence of normal blood glucose level

18 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 18 Children and HIV HIV infections in children HIV infections in children Perinatally through the placenta Perinatally through the placenta Exposure to infected maternal blood and vaginal secretions Exposure to infected maternal blood and vaginal secretions Postpartum ingestion of breast milk Postpartum ingestion of breast milk

19 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 19 Cerebrovascular Disease Cerebrovascular disease in children differs in adults in three ways Cerebrovascular disease in children differs in adults in three ways Absence of predisposing factors Absence of predisposing factors Differences in the clinical response Differences in the clinical response Anatomic site of the pathologic condition Anatomic site of the pathologic condition Occlusive cerebrovascular disease Occlusive cerebrovascular disease Hemorrhagic cerebrovascular disease Hemorrhagic cerebrovascular disease

20 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 20 Childhood Tumors Most common solid tumor and second most common primary neoplasm Most common solid tumor and second most common primary neoplasm Primary is leukemia Primary is leukemia ~50% of solid tumors are nonmalignant ~50% of solid tumors are nonmalignant Account for 20% of all childhood cancers Account for 20% of all childhood cancers Annual incidence of 2.4 to 4 per 100,000 Annual incidence of 2.4 to 4 per 100,000 2000 cases are diagnosed each year 2000 cases are diagnosed each year The leading cause of death from disease in children 1 to 15 years The leading cause of death from disease in children 1 to 15 years

21 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 21 Childhood Tumors Brain tumors Brain tumors Medulloblastoma Medulloblastoma Ependymoma Ependymoma Astrocytoma Astrocytoma Brainstem glioma Brainstem glioma Optic nerve glioma Optic nerve glioma

22 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 22 Childhood Brain Tumors

23 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 23 Childhood Tumors Embryonal tumors Embryonal tumors Neuroblastoma (aggressive tumor) Neuroblastoma (aggressive tumor) Originates in neural crest cells (develop into sympathetic nervous system) Originates in neural crest cells (develop into sympathetic nervous system) Most diagnosed during first 2 years Most diagnosed during first 2 years 75% found before child is 5 years 75% found before child is 5 years Abdomen (65%) most often in the adrenal medulla Abdomen (65%) most often in the adrenal medulla Mediastinum (15%) Mediastinum (15%)

24 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 24 Childhood Tumors Embryonal tumors Embryonal tumors Retinoblastoma (rare congenital eye tumor) Retinoblastoma (rare congenital eye tumor) Rarely diagnosed after age 5 Rarely diagnosed after age 5 40% inherited as autosomal dominant disorder 40% inherited as autosomal dominant disorder Prognosis for most children is excellent, with a greater than 90% long-term survival Prognosis for most children is excellent, with a greater than 90% long-term survival Children with bilateral or metastatic disease have a poor prognosis Children with bilateral or metastatic disease have a poor prognosis About 75% have useful vision in the treated eye About 75% have useful vision in the treated eye

25 Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 25 Childhood Tumors


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