Ch 20: Alterations of Neurologic Function in Children
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Questions and Answers

Which of the following is a primary characteristic of dyskinetic cerebral palsy?

  • Gait disturbances and instability due to cerebellar damage.
  • Extreme difficulty in fine motor coordination and purposeful movements. (correct)
  • Spasticity primarily affecting the lower extremities.
  • Severe hypotonia at birth followed by trunk stiffness.

    • A child presents with gait disturbances, instability, and initial hypotonia that progresses to trunk stiffness. Which type of cerebral palsy is MOST likely?

  • Spastic cerebral palsy
  • Mixed-variety cerebral palsy
  • Ataxic cerebral palsy (correct)
  • Dyskinetic cerebral palsy

    • What is the underlying cause of phenylketonuria (PKU)?

  • Viral infection affecting amino acid metabolism.
  • Autosomal recessive mutation affecting phenylalanine hydroxylase (PAH). (correct)
  • Dietary deficiency of essential amino acids.
  • Excessive production of tyrosine.

    • What is the primary pathophysiology of PKU that leads to CNS damage?

    <p>Accumulation of phenylalanine and associated metabolites, causing neural damage. (B)</p> Signup and view all the answers

    Why is newborn screening crucial for PKU?

    <p>To rapidly detect and prevent progressive developmental delay. (D)</p> Signup and view all the answers

    In which condition does a sac-like cyst containing meninges protrude through a defect in the posterior arch of the vertebrae, but the spinal cord remains intact?

    <p>Meningocele (B)</p> Signup and view all the answers

    An infant is born with a midline skull defect and a protruding mass in the occipital area. Which condition is MOST likely associated with this presentation?

    <p>Occipital Encephalocele (D)</p> Signup and view all the answers

    Which of the following is MOST characteristic of myelomeningocele?

    <p>It involves cystic dilation of meninges and protuberance of the spinal cord through a vertebral defect. (A)</p> Signup and view all the answers

    A newborn is diagnosed with an encephalocele that extends into the orbit of the eye. Which of the following clinical manifestations is MOST likely to be observed?

    <p>Proptosis on the affected side (D)</p> Signup and view all the answers

    Cerebral palsy is defined as which of the following?

    <p>A disorder of movement, muscle tone, or posture due to brain injury or abnormal development. (C)</p> Signup and view all the answers

    A child with cerebral palsy exhibits increased muscle tone, exaggerated deep tendon reflexes, and clonus. Which type of cerebral palsy is MOST likely present?

    <p>Pyramidal (spastic) cerebral palsy (D)</p> Signup and view all the answers

    Which prenatal factor is LEAST likely to contribute to the development of cerebral palsy?

    <p>Advanced maternal age (C)</p> Signup and view all the answers

    A child presents with hemiparetic cerebral palsy following a perinatal stroke. Which vascular territory was MOST likely affected by the stroke?

    <p>Middle cerebral artery (C)</p> Signup and view all the answers

    Which intervention is MOST directly aimed at reducing spasticity in children with cerebral palsy?

    <p>Intrathecal baclofen pump (C)</p> Signup and view all the answers

    Damage to the pyramidal system during brain development is MOST likely to result in which type of cerebral palsy?

    <p>Spastic (C)</p> Signup and view all the answers

    Which of the following is a common clinical manifestation associated with cerebral palsy, beyond motor deficits?

    <p>Seizures (B)</p> Signup and view all the answers

    A newborn is diagnosed with cerebral palsy. Which perinatal event is MOST likely to be a contributing factor?

    <p>Trauma during birth (B)</p> Signup and view all the answers

    Which of the following is MOST characteristic of spastic quadriparesis cerebral palsy?

    <p>Spasticity throughout the entire body (B)</p> Signup and view all the answers

    A child with spastic cerebral palsy is undergoing an evaluation. Which finding would suggest involvement of the corticospinal tracts?

    <p>Increased muscle tone and exaggerated reflexes (B)</p> Signup and view all the answers

    Which intervention focuses on improving functional communication skills for a child with cerebral palsy?

    <p>Speech therapy (B)</p> Signup and view all the answers

    What dietary modification is MOST essential for managing phenylketonuria (PKU)?

    <p>Restricting phenylalanine intake (D)</p> Signup and view all the answers

    A newborn screening reveals elevated phenylalanine levels. What is the MOST important next diagnostic step?

    <p>Performing a quantitative amino acid analysis (B)</p> Signup and view all the answers

    What is the anticipated outcome of an infant who is diagnosed with PKU at birth but receives appropriate dietary treatment?

    <p>Normal cognitive development (C)</p> Signup and view all the answers

    During which gestational week does the neural groove typically close to form the neural tube?

    <p>Fourth (C)</p> Signup and view all the answers

    Deficiency of which nutrient during pregnancy is MOST strongly associated with neural tube defects?

    <p>Folate (D)</p> Signup and view all the answers

    Which type of neural tube defect is generally considered the MOST common?

    <p>Posterior (A)</p> Signup and view all the answers

    In a pregnant woman, elevated amniotic fluid alpha-fetoprotein (AFP) levels might indicate the presence of what condition in the fetus?

    <p>Neural tube defect (A)</p> Signup and view all the answers

    What is a key characteristic of dyskinetic cerebral palsy that differentiates it from other types?

    <p>Jerky, uncontrolled movements primarily affecting fine motor skills (D)</p> Signup and view all the answers

    What should a healthcare provider immediately assess in a baby presenting with a bulging fontanel and macrocephaly?

    <p>Head circumference and compare to standard growth charts (C)</p> Signup and view all the answers

    Which area of the brain is primarily affected in a patient with ataxic cerebral palsy, leading to gait and stability issues?

    <p>Cerebellum (A)</p> Signup and view all the answers

    Microcephaly can arise from a variety of factors affecting brain development. Which of the following is LEAST likely to be a direct cause of microcephaly?

    <p>Prenatal physical trauma to the mother (A)</p> Signup and view all the answers

    What specific enzymatic defect causes individuals with phenylketonuria (PKU) to require a special diet?

    <p>Inability to convert phenylalanine into tyrosine (B)</p> Signup and view all the answers

    The term myelodysplasia broadly refers to defects in the development of the spinal cord. Which of the following conditions falls under the umbrella of myelodysplasia?

    <p>Spina bifida (A)</p> Signup and view all the answers

    Which dietary modification is MOST critical for managing phenylketonuria (PKU) to prevent neurological damage?

    <p>Restriction of phenylalanine intake (C)</p> Signup and view all the answers

    Why do uncontrolled levels of phenylalanine in PKU patients cause significant neurological damage?

    <p>High phenylalanine levels competitively inhibit transport of other amino acids into the brain. (A)</p> Signup and view all the answers

    A child with cerebral palsy is undergoing an evaluation. Which assessment would provide the MOST direct information about the severity of spasticity?

    <p>Modified Ashworth Scale (A)</p> Signup and view all the answers

    Which of the following dietary modifications is MOST critical for managing phenylketonuria (PKU)?

    <p>Restricting phenylalanine intake while ensuring adequate tyrosine (A)</p> Signup and view all the answers

    What is the primary goal of early intervention programs for infants diagnosed with cerebral palsy?

    <p>Prevent the development of associated impairments and maximize function (B)</p> Signup and view all the answers

    What is indicated when a child with cerebral palsy has increased muscle tone, hyperactive reflexes, and clonus?

    <p>Spastic cerebral palsy (C)</p> Signup and view all the answers

    Why is it important to monitor tyrosine levels in individuals with phenylketonuria (PKU)?

    <p>To prevent tyrosine deficiency due to reduced phenylalanine conversion. (A)</p> Signup and view all the answers

    Which congenital defect involves incomplete closure of the vertebral column without herniation of the meninges or spinal cord?

    <p>Spina bifida occulta (C)</p> Signup and view all the answers

    Which type of cerebral palsy involves injury to the basal ganglia, resulting in abnormal, involuntary movements, and difficulty with fine motor skills?

    <p>Dystonic (B)</p> Signup and view all the answers

    Flashcards

    Encephalocele

    Herniation of brain and meninges through a skull defect, forming a sac.

    Spina Bifida

    Incompletely formed posterior vertebral arch allowing spinal cord protrusion.

    Meningocele

    Cyst-like dilation of meninges protruding through vertebral arch, spinal cord intact.

    Myelomeningocele

    Cystic dilation of meninges with spinal cord protrusion, more severe complications.

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    Cerebral Palsy

    A movement disorder caused by brain injury or abnormal development, usually before age 1.

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    Extrapyramidal cerebral palsy

    Cerebral palsy caused by damage to the basal ganglia, thalamus, or cerebellum, leading to movement disorders.

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    Dyskinetic cerebral palsy

    A subtype of extrapyramidal CP with jerky, uncontrolled movements due to basal ganglia or thalamus damage.

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    Ataxic cerebral palsy

    A subtype of extrapyramidal CP characterized by gait disturbances and instability, linked to cerebellum damage.

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    Phenylketonuria (PKU)

    An autosomal recessive disorder causing phenylalanine buildup due to PAH gene mutations, affecting amino acid metabolism.

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    Phenylalanine hydroxylase (PAH)

    An enzyme involved in converting phenylalanine to tyrosine; its deficiency leads to PKU development.

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    Pathophysiology

    The study of the functional changes associated with a disease or injury.

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    Risk Factors for Cerebral Palsy

    Factors like low birth weight, asphyxia, and prenatal issues that increase CP likelihood.

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    Spastic Cerebral Palsy

    A type of CP caused by damage to corticospinal pathways, leading to increased muscle tone.

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    Pyramidal System

    Neural pathways controlling voluntary movement, affected in pyramidal cerebral palsy.

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    Clinical Manifestations of CP

    Symptoms like vision/hearing impairment, seizures, and intellectual disability can occur.

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    Hemiparetic Cerebral Palsy

    A form of CP where one side of the body is affected, often after a stroke.

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    Management of Spasticity

    Treatments like baclofen pumps and botulinum toxin improve spasticity in CP patients.

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    C-reactive protein

    A substance produced by the liver in response to inflammation.

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    Neural tube defect (NTD)

    A birth defect resulting from incomplete closure of the neural tube.

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    Microcephaly causes

    Conditions leading to abnormal small head size, often genetic or environmental.

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    Myelodysplasia

    A defect in spinal cord formation that can also refer to brain anomalies.

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    Autosomal gene alterations

    Genetic changes that can lead to conditions like microcephaly.

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    Extrapyramidal tracts

    Neural pathways affecting involuntary movements, vital for coordination.

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    Prenatal physical abuse effects

    Can cause various developmental issues, including microcephaly in infants.

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    Hydrocephalus

    A condition characterized by an accumulation of cerebrospinal fluid, leading to increased intracranial pressure and macrocephaly.

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    PKU

    Phenylketonuria is an inherited disorder where the body cannot break down phenylalanine, leading to a toxic buildup.

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    Phenylalanine to Tyrosine

    Children with PKU cannot convert phenylalanine into tyrosine due to a deficiency of phenylalanine hydroxylase.

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    Benign Febrile Seizures

    Seizures occurring in children associated with fever, typically above 38.5°C (101.3°F) without serious underlying issues.

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    Gait Disturbances

    Impaired walking patterns often observed in conditions like ataxic cerebral palsy.

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    Measurement of Head Circumference

    A critical assessment in infants to monitor for conditions like hydrocephalus by comparing to growth charts.

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    Lumbar Puncture

    A medical procedure to collect cerebrospinal fluid, useful for assessing infections in cases like hydrocephalus.

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    Neural Groove Closure Week

    The neural groove closes dorsally during the fourth week of gestation, forming the neural tube which becomes the CNS.

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    Folate Supplementation

    Folate is crucial for pregnant women to prevent neural tube defects like meningocele.

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    Common Neural Tube Defect

    The most common defect of neural tube closure is posterior defects, affecting spinal development.

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    Anencephaly

    Anencephaly is a severe condition where much of the brain and skull is missing.

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    Obstructive Hydrocephalus Cause

    The most common cause of obstructive hydrocephalus in infants is stenosis of the aqueduct of Sylvius.

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    CNS Formation

    The neural tube, formed from the neural groove, develops into the central nervous system.

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    Maternal Folate Deficiency

    Deficiency of folate in mothers can lead to neural tube defects, notably meningocele.

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    Hydrocephalus Growth Cause

    Hydrocephalus results from increased cerebrospinal fluid (CSF) leading to head enlargement.

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    Study Notes

    Alterations of Neurologic Function in Children

    • Neural Tube Formation: Neural tube formation begins between 3 and 4 weeks gestation. The neural plate folds to form a neural groove and then neural folds, which further develop into the neural tube. This tube forms the central nervous system (CNS).
    • Neural Tube Closure: Closure of the neural tube occurs first in the cervical region, progressing cranially and caudally.

    Neural Tube Defects

    • Categories: Neural tube defects (NTDs) are categorized as anterior midline defects (ventral induction) and posterior defects (dorsal induction).
      • Anterior Midline Defects: May cause brain and facial abnormalities, such as those seen in holoprosencephaly spectrum disorders.
      • Posterior Defects: Dorsal induction defects are the most common type, resulting in various myelodysplasias. Myelodysplasia encompasses a range of vertebral and spinal cord anomalies.
    • Specific Defects:
      • Encephalocele: A herniation or protrusion of brain and meninges through a defect in the skull. May involve the nasopharynx.
      • Meningocele: A sac-like cyst of meninges filled with spinal fluid through a vertebral defect. The spinal cord remains intact.
      • Spina Bifida: A failure of the posterior vertebral arches to fuse. Includes spina bifida occulta (less severe, often without visible defects).
      • Myelomeningocele: A saclike cyst containing meninges, spinal fluid, and a portion of the spinal cord and nerves. The spinal cord is exposed.
      • Spina Bifida Occulta: A less severe form with no visible external defects, but internal abnormalities are possible.

    Anencephaly

    • Definition: A birth defect where parts of the brain and skull are missing.
    • Symptoms/Prognosis: Typically fatal shortly after birth due to severe brain damage. Can sometimes be detected prenatally (ultrasound) or through analyzing maternal serum a-fetoprotein (AFP).

    Additional Information

    • Maternal Folate Deficiency: Both meningocele and myelomeningocele are linked to maternal folate deficiency.
    • Timing of Defects: Neural tube defects occur during the first few weeks of pregnancy.
    • Encephalocele Location: Encephalocele defects can occur in different areas of the head (frontal, occipital,etc).

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    Related Documents

    Chapter 20 Alterations of Neurologic Function in Children PDF
    Chapter 20: Alterations of Neurologic Function in Children PDF

    Description

    This quiz explores alterations in neurologic function in children, focusing on neural tube formation, closure, and defects. You will learn about the categories of neural tube defects and their implications for brain and spinal cord anomalies. Test your knowledge on the critical stages of neural development and the outcomes of related disorders.

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