PEDS Exam 5 Modules 11, 12, 13
Module 11 Neurologic Disorders
Anatomy & Physiology
Peripheral Nervous System
o Somatic/Voluntary system: afferent fibers carry info to brain, efferent fibers carry info to body
o Autonomic/Involuntary system: Sympathetic system responsible for flight or fight, Parasympathetic responsible for rest
and recuperation
Central Nervous System: brain and spinal cord
Reflexes:
o Primitive: absent = CNS problem, persistent indicates frontal lobe problem
o Deep tendon: decreased response = PNS problem, Exaggerated response = CNS problem
Assessment
General history: birth history, diagnoses, surgeries, accidents, illnesses, milestones, behavioral issues, activity level, parenting
variations, socioeconomic issues
Physical exam: microcephaly or macrocephaly, hypertonia or hypotonia, muscle wasting/atrophy, skin lesions, dysmorphic
features, CN abnormalities, developmental screening, motor skills
Diagnosis: chromosome testing, metabolic screening, thyroid testing, MRI
Treatment: multidisciplinary approach, treat associated conditions
Spina Bifida
Most common neural tube defect
Myelomeningocele Section of spinal cord and associated nerves are exposed and visible on
(Spina Bifida Cystica) outside of body
Causes partial or complete paralysis before opening
Can be removed surgically, doesn’t correct deficits
Cover defect with saline-soaked sterile gauze
Meningocele Membrane that surrounds spinal cord is enlarged creating a sac
Sac present on back contains meninges, may not be overly visible on exam
Doesn’t always cause disability
Can be surgically removed, doesn’t correct deficits
Spina Bifida Occulta Section of spinal vertebrae is malformed, but cord and nerves are normal
Not visible, may have dimple, hair, or hemangioma in sacral area
Common version is tethered cord syndrome where tissue attachments
limit movement of spinal cord within the column
Treatment Cover defect with a sterile dressing soaked in warm sterile NS, position prone with hips flexed and
lying on open diaper
Baseline OFC and continued monitoring (risk for hydrocephalus, increased ICP)
Antibiotics to prevent infection
Immediately consult pediatric neurosurgery
Will need consult and follow up with neurosurgery, neurology, PT/OT/ST, social work, child life
specialist
Prone with hip flex when admitting
Education All females of child-bearing age should take folic acid supplements to prevent spina bifida, usually develops
before most women know they are pregnant
Anencephaly & Encephalocele
, Anencephaly Neural cord defect where majority of brain doesn’t develop
Can be missing both portions of brain and skull
Usually stillborn or die shortly after birth
Rate has decreased by 28% since US started fortifying grains with folic acid
(3 in every 10000 pregnancies)
Encephalocele Portion of brain protrudes through an opening in the skull
Severity depends on size and location
o Groove in middle of skull
o Between forehead and nose
o On back of skull
Chiari Malformation
Structural defect in cerebellum, where bony space at lower portion of skull is smaller than normal, cerebellum and brainstem are
pushed downward and can block flow of CSF
Type 1: usually no symptoms, Type 2: “Arnold-Chiari Type 3: Associated with Type 4: “Cerebellar
diagnosed by accident Malformation” larger occipital encephalocele hypoplasia” cerebellum in
downward herniation of correct position, but parts are
cerebellum through foramen missing, usually do not survive
magnum to spinal cord, may infancy
accompany
myelomeningocele
Symptoms Dizziness, weakness, numbness, vision problems, HA, balance and coordination problems
Potential for cyst to form on spinal column (syrinx or syringomyelia) causing numbness in the
lower extremities
Diagnosis Brain MRI will show protrusion of cerebellum
Treatment Type 1: no treatment usually
Type 2: may require treatment depending on degree of protrusion, decompression
Type 3 & 4: may require surgery to decompress cerebellum & create more space to minimize s/s
Education Prevent injury and no contact sports
Congenital Malformations
Agenesis of corpus callosum: structure that connects 2 hemispheres of brain is partially or completely missing
o Most common brain malformation
o Can range from normal intelligence with mild learning deficiencies to severe mental retardation
o Can occur alone or with other defects
Microcephaly: OFC is more than 2 standard deviations below normal, brain has not developed properly or stopped growing,
may be present at birth or develop later, often causes delays
o Associated with Down syndrome, chromosomal abnormalities, maternal substance abuse, maternal CMV, Zika virus,
rubella, chicken pox exposure
o Can cause dysmorphic facial features, growth retardation, hyperactivity, and seizures
o Supportive care, refer to early intervention
Craniosynostosis: premature closure of sutures in skill that results in abnormal head shape
o Often associated with facial deformities
o Surgical correction performed in infancy
Hydrocephalus
Module 11 Neurologic Disorders
Anatomy & Physiology
Peripheral Nervous System
o Somatic/Voluntary system: afferent fibers carry info to brain, efferent fibers carry info to body
o Autonomic/Involuntary system: Sympathetic system responsible for flight or fight, Parasympathetic responsible for rest
and recuperation
Central Nervous System: brain and spinal cord
Reflexes:
o Primitive: absent = CNS problem, persistent indicates frontal lobe problem
o Deep tendon: decreased response = PNS problem, Exaggerated response = CNS problem
Assessment
General history: birth history, diagnoses, surgeries, accidents, illnesses, milestones, behavioral issues, activity level, parenting
variations, socioeconomic issues
Physical exam: microcephaly or macrocephaly, hypertonia or hypotonia, muscle wasting/atrophy, skin lesions, dysmorphic
features, CN abnormalities, developmental screening, motor skills
Diagnosis: chromosome testing, metabolic screening, thyroid testing, MRI
Treatment: multidisciplinary approach, treat associated conditions
Spina Bifida
Most common neural tube defect
Myelomeningocele Section of spinal cord and associated nerves are exposed and visible on
(Spina Bifida Cystica) outside of body
Causes partial or complete paralysis before opening
Can be removed surgically, doesn’t correct deficits
Cover defect with saline-soaked sterile gauze
Meningocele Membrane that surrounds spinal cord is enlarged creating a sac
Sac present on back contains meninges, may not be overly visible on exam
Doesn’t always cause disability
Can be surgically removed, doesn’t correct deficits
Spina Bifida Occulta Section of spinal vertebrae is malformed, but cord and nerves are normal
Not visible, may have dimple, hair, or hemangioma in sacral area
Common version is tethered cord syndrome where tissue attachments
limit movement of spinal cord within the column
Treatment Cover defect with a sterile dressing soaked in warm sterile NS, position prone with hips flexed and
lying on open diaper
Baseline OFC and continued monitoring (risk for hydrocephalus, increased ICP)
Antibiotics to prevent infection
Immediately consult pediatric neurosurgery
Will need consult and follow up with neurosurgery, neurology, PT/OT/ST, social work, child life
specialist
Prone with hip flex when admitting
Education All females of child-bearing age should take folic acid supplements to prevent spina bifida, usually develops
before most women know they are pregnant
Anencephaly & Encephalocele
, Anencephaly Neural cord defect where majority of brain doesn’t develop
Can be missing both portions of brain and skull
Usually stillborn or die shortly after birth
Rate has decreased by 28% since US started fortifying grains with folic acid
(3 in every 10000 pregnancies)
Encephalocele Portion of brain protrudes through an opening in the skull
Severity depends on size and location
o Groove in middle of skull
o Between forehead and nose
o On back of skull
Chiari Malformation
Structural defect in cerebellum, where bony space at lower portion of skull is smaller than normal, cerebellum and brainstem are
pushed downward and can block flow of CSF
Type 1: usually no symptoms, Type 2: “Arnold-Chiari Type 3: Associated with Type 4: “Cerebellar
diagnosed by accident Malformation” larger occipital encephalocele hypoplasia” cerebellum in
downward herniation of correct position, but parts are
cerebellum through foramen missing, usually do not survive
magnum to spinal cord, may infancy
accompany
myelomeningocele
Symptoms Dizziness, weakness, numbness, vision problems, HA, balance and coordination problems
Potential for cyst to form on spinal column (syrinx or syringomyelia) causing numbness in the
lower extremities
Diagnosis Brain MRI will show protrusion of cerebellum
Treatment Type 1: no treatment usually
Type 2: may require treatment depending on degree of protrusion, decompression
Type 3 & 4: may require surgery to decompress cerebellum & create more space to minimize s/s
Education Prevent injury and no contact sports
Congenital Malformations
Agenesis of corpus callosum: structure that connects 2 hemispheres of brain is partially or completely missing
o Most common brain malformation
o Can range from normal intelligence with mild learning deficiencies to severe mental retardation
o Can occur alone or with other defects
Microcephaly: OFC is more than 2 standard deviations below normal, brain has not developed properly or stopped growing,
may be present at birth or develop later, often causes delays
o Associated with Down syndrome, chromosomal abnormalities, maternal substance abuse, maternal CMV, Zika virus,
rubella, chicken pox exposure
o Can cause dysmorphic facial features, growth retardation, hyperactivity, and seizures
o Supportive care, refer to early intervention
Craniosynostosis: premature closure of sutures in skill that results in abnormal head shape
o Often associated with facial deformities
o Surgical correction performed in infancy
Hydrocephalus
