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Pathopharm Exam 3 > Module 7: Part 4 (120-158) > Flashcards

Module 7: Part 4 (120-158) Flashcards

1
Q

The skull and much of the brain is missing

A

Anencephaly

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2
Q

What is Encephalocele?

A

A saclike herniation of various amounts of brain and meninges protrude through a defect in the skull; requires surgery to correct.

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3
Q

Is a saclike cyst of meninges filled with spinal fluid.

A

Meningocele

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4
Q

Myelomeningocele contains _____

A

Contains neural tissue, spinal cord, or nerves.

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5
Q

Cortical Dysplasia is caused by______

A

defects in neuronal cell migration and subsequent abnormalities in connections between cells.

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6
Q

Microcephaly

A

Small brain is caused by reduced proliferation or accelerated apoptosis.
Is not treatable.
Can be true (primary) or secondary.

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7
Q

Cranial deformities or craniostenosis
is the Premature closure of one or more of the cranial sutures during the first _______ months of life

A

18 to 20

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8
Q

What are the 2 Nonsyndromic craniosynostosis?

A

Simple craniosynostosis: Only one suture is involved.
Compound or complex craniosynostosis: Two or more sutures are involved.

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9
Q

What is an Imbalance between the production and reabsorption of cerebrospinal fluid (CSF)?

A

Congenital hydrocephalus

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10
Q

What are the 2 big clinical changes of congenital hydrocephalus?

A

Ventricular enlargement and increased intracranial pressure
Dilates the ventricles and pushes or compresses brain tissue against the skull.

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11
Q

Obstructive hydrocephalus:

A

Congenital aqueductal stenosis

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12
Q

Dandy-Walker malformation:

A

Blind pouch

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13
Q

What are the S/S of congenital hydrocephalus?

A

Enlarged fontanels that become full and bulging
Sunsetting eyes
Inability to hold the head upright
Dramatic head growth

If increased intracranial pressure develops
High pitched cry; irritability, lethargy, and vomiting

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14
Q

if increased ICP develops with congenital hydrocephalus what s/s would you see?

A

High pitched cry; irritability, lethargy, and vomiting

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15
Q

What is the tx for congenital hydrocephalus?

A

shunt insertion

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16
Q

Normal vs. Hydrocephalus brain anatomy

A
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17
Q

Nonprogressive syndromes that cause motor deficits =

A

cerebral palsy

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18
Q

what are the causes of cerebral palsy?

A

Prenatal cerebral hypoxia
Perinatal or postnatal trauma
Chromosomal aberrations
Radiation
Maternal dibetes
Etc………

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19
Q

Pryamidal or spastic cerebral palsy

A

Damage or defects in the brain’s corticospinal pathways
Spastic quadriparesis versus hemiparetic cerebral palsy

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20
Q

Extrapryamidal or nonspastic cerebral palsy is a/w damage to cells where?

A

Damage to cells in the basal ganglia, thalamus, or cerebellum
Dyskinetic cerebral palsy versus ataxic cerebral palsy

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21
Q

What is the treatment for cerebral palsy?

A

Magnesium sulfate, cooling head, family-focused multidisciplinary team approach

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22
Q

Inherited Metabolic Disorders of the Central Nervous System: Defects in amino acid metabolism include what 2 disorders?

A

Phenylketonuria (PKU)
Hyperphenylalaninemia

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23
Q

Phenylketonuria (PKU) involves mutations of the ______ gene

A

phenylalanine hydroxylase (PAH)

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24
Q

What is the treatment for PKU?

A

Restriction of phenylalanine in the diet
Equal (Aspartame)

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25
Q

Tay-Sachs disease

A

Accumulation of gangliosides (gangliosidosis), causing toxicity to nerve cells
Death usually occurs by 2 to 5 years of age.
No beneficial therapy is available.

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26
Q

Death usually occurs by ___ to ___ years of age with Tay-Sachs disease

A

2-5

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27
Q

Lysosomal storage diseases result in _____

A

altered lipid metabolism

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28
Q

Acute encephalopathies r/t drugs typically occur due to:

A

Accidental ingestion, therapeutic overdose, or intentional overdose

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29
Q

If not treated, causes serious and irreversible neurologic damage

A

lead poisoning

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30
Q

Who is at greatest risk for lead poisoning?

A

Children 2 to 3 years of age and those with pica (habitual, purposeful, and compulsive ingestion of nonfood substances such as clay, dirt, or paint chips) or children living in lead-contaminated environments.

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31
Q

What is meningitis?

A

Inflammation of the meninges and subarachnoid space surrounding the brain and spinal cord; encephalitis—inflammation within the brain

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32
Q

Aseptic meningitis has no evidence of _____

A

bacterial infection

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33
Q

Approximately 4100 cases are reported each year; one-half of the cases are children younger than 18 years of age.

A

bacterial meningitis

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34
Q

Vaccines have reduced the number of incidences of bacterial meningitis T/F

A

TRUE

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35
Q

What is bacterial meningitis often preceeded by?

A

Often preceded by an upper respiratory or a gastrointestinal infection

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36
Q

Fever, headache, vomiting, and irritability are characteristic of which meningitis?

A

bacterial

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37
Q

What are the CNS symptoms a/w bacterial meningitis?

A

Photophobia
Nuchal and spinal rigidity
Decreased level of consciousness
Seizures

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38
Q

Meningococcal meningitis can produce a characteristic ______ rash.

A

petechial

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39
Q

Bacterial meningitis treatment includes ___ and possibly ____

A

antibiotics and possibly steroids.

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40
Q

Infants may have buldging fontanels with bacterial meningitis T/F

A

TRUE

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41
Q

Irritation of the meninges and spinal roots a/w bacterial meningitis causes what s/s?

A

Nuchal rigidity
Positive Kernig sign
Positive Brudzinski sign
Opisthotonic posturing

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42
Q

What is a hallmark of viral meningitis?

A

Hallmark is a mononuclear response in the CSF and the presence of normal blood glucose level.

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43
Q

Which has milder clinical manifestations: bacterial or viral meningitis?

A

viral

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44
Q

Treatment of viral meningitis includes antibiotics and antiviral medications. T/F

A

TRUE

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45
Q

Human immunodeficiency virus–type 1(HIV-1) causes a syndrome called _____ in infants and children

A

HIV-1 encephalopathy

46
Q

What is the treatment of HIV-1 in infants and children?

A

Aggressive response to opportunistic infections
Support and relief of symptomatic occurrences
Administration of highly active antiretroviral therapy

47
Q

What might you see with perinatal strokes in children?

A

Not moving one side of the body, handedness before normal, dragging foot, contralateral hemiplegia

48
Q

_____ strokes are Rare and usually no signs

A

ischemic

49
Q

hemorrhagic strokes are usually from _____

A

vascular anomalies that rupture; Moyamoya disease

50
Q

what is stroke treatment?

A

Surgery, radiation therapy, and embolic occlusion of malformation

51
Q

Epilepsy is a/w Dysregulation of inhibitory gamma-aminobutyric acid B (GABAB) neurotransmission T/F

A

FALSE
GABA-A

52
Q

What is epilepsy?

A

Occurrence of seizures
Abnormal discharge of electrical activity in the brain
Dysregulation of inhibitory gamma-aminobutyric acid A (GABAA) neurotransmission

53
Q

partial seizures suggest more localized brain dysfunction T/F

A

TRUE

54
Q

Unclassified epileptic seizures present how?

A

Rhythmic eye movements, chewing, and swimming movements

55
Q

nonconvulsive activity is a/w _____ seizures

A

absence

56
Q

convulsive activity includes ___, ___, or ____ activity

A

tonic-tonic, tonic, or clonic activity

57
Q

Treatment of childhood absence epilepsy includes : Antiepileptic drugs and ________ diet

A

ketogenic

58
Q

Childhood absence epilepsy is aka ___ or ______

A

Petit mal seizures or nonconvulsive epilepsy

59
Q

epilepsy generalized seizures can include convulsive activity or nonconvulsive activity T/F

A

TRUE

60
Q

Epilepsy syndromes are/characterize what? What are some examples?

A

Are seizure disorders that display a group of signs and symptoms that collectively occur.
Characterize or indicate a particular condition.
Examples
Febrile seizures
Infantile spasms
Lennox-Gastaut syndrome
Juvenile myoclonic epilepsy

61
Q

Temperatures higher than ______º F can cause simple febrile seizures.

A

102.2

62
Q

What age do simple febrile seizures often occur?

A

Often occur between the ages of 9 months and 5 years.

63
Q

What is the recommended treatment for simple febrile seizures?

A

Phenobarbital use is recommended.

64
Q

What is the most common and benign epilepsy syndrome?

A

simple febrile seizures

Are from a high temperature.
Afflict 2% to 5% of children.

65
Q

Have a longer duration than benign febrile seizures, usually longer than 15 minutes.

A

complex febrile seizures

66
Q

Complex febrile seizures have ____ characteristics

A

focal

67
Q

With complex febrile seizures usually only 1 seizure occurs in a 24 hr period T/F

A

FALSE
Usually more than one seizure occurs in a 24-hour period.

68
Q

Is considered a risk factor for the development of epilepsy

A

complex febrile seizures

69
Q

Commonly referred to as jackknife seizures

A

Infantile spasms (West syndrome)

70
Q

Onset for infantile spasms (West syndrome) is usually between 1 and 2 months of age. T/F

A

FALSE
Onset is usually between 4 and 8 months of age.

71
Q

Why might infantile spasms (West syndrome) occur?

A

May be idiopathic or may occur in response to a CNS insult or from tuberous sclerosis complex.

72
Q

Usually happen in clusters and occur 5 to 150 times per day

A

Infantile spasms (West syndrome)

73
Q

What is the treatment for Infantile spasms (West syndrome)?

A

High doses of oral steroids are recommended.

74
Q

Lennox-Gastaut syndrome

A

Is characterized by an onset of seizures early in childhood.
Occurs in male infants and children more often than female infants and children.
Begins between 1 and 8 years of age.
Includes a variety of generalized seizures.
Treatment is difficult: Includes multiple medications and alternate therapies of a ketogenic diet or a vagal nerve stimulator; may need epilepsy surgery.

75
Q

Is a primary, generalized epilepsy that usually affects adolescents and young adults.

A

Juvenile myoclonic epilepsy

76
Q

Juvenile myoclonic epilepsy treatment includes medications prescribed for 7 weeks T/F

A

FALSE
for life!

77
Q

Is a benign form of epilepsy that involves myoclonic jerks of the neck, shoulders, and arms, as well as generalized tonic-clonic seizures.

A

Juvenile myoclonic epilepsy
Can be single or repetitive

78
Q

What should be included in the evaluation for epilepsy?

A

Electroencephalography (EEG)
Magnetic resonance imaging (MRI)
History
Clinical presentation

79
Q

What all is included in the treatment of epilepsy?

A

Anticonvulsants
Ketogenic diet
Surgery
Vagal nerve stimulator

80
Q

With continuing or recurring seizure activity recovery from seizure activity is incomplete. T/F

A

TRUE

81
Q

Continuing or recurring seizure activity
Usually lasts for ______ minutes or longer.

A

30

82
Q

Any seizure activity can evolve into status epilepticus. T/F

A

TRUE

83
Q

Continuing or recurring seizure activity
is a medical emergency that requires immediate intervention. T/F

A

TRUE

84
Q

Are the most common solid tumor and second most common primary neoplasm.

A

Brain tumors

85
Q

______ is the most common primary neoplasm.

A

Leukemia

86
Q

Approximately ____% of solid tumors are nonmalignant.

A

45

87
Q

Account for 20% of all childhood cancers.

A

brain tumors

88
Q

Primary brain tumors arise from brain (glial) tissue and do not metastasize. T/F

A

TRUE

89
Q

Is the leading cause of death from disease in children 1 to 15 years of age.

A

Brain tumors

90
Q

Two-thirds of all pediatric brain tumors are found in the _______ region of the brain.

A

posterior fossa

This area may be called the infratentorial region because of its location below the tentorium, which is the layer of dura mater that separates the cerebellum from the hemispheres or cerebrum.

91
Q

Approximately one-third of childhood tumors are located above the ______.

A

tentorium
Called the supratentorial region

92
Q

Is the most common childhood malignant tumor; this invasive tumor is fast growing; angiogenesis is the hallmark.

A

Medulloblastoma

93
Q

Develops in the fourth ventricle, cysts, and calcifications.

A

Ependymoma

94
Q

Astrocytoma causes (unilateral/bilateral) symptoms.

A

Causes unilateral (occurring on the same side) symptoms.

95
Q

What occurs with a Brainstem glioma?

A

Unilateral paralysis of cranial nerves occurs with contralateral paralysis of the arm and leg, hyperreflexia, and extensor plantar responses

96
Q

Optic nerve glioma

A

May cause complete unilateral blindness and hemianopia of the other eye.

97
Q

Craniopharyngioma

A

Originates from the pituitary gland or the hypothalamus; is usually slow growing and may be quite large when diagnosed.

98
Q

If the fourth ventricle is blocked, then hydrocephalus and signs of increased intracranial pressure are exhibited, includings:

A

Headache is worse in the morning.
Vomiting occurs in the morning.

99
Q

Infratentorial tumors exhibit localized signs of impaired_____ and _____

A

coordination and balance.

100
Q

What should be included in the evaluation of childhood tumors?

A

Complete workup
Computed tomography (CT) with contrast
MRI
Magnetic resonance angiography (MRA)
Lumbar puncture

101
Q

What should be included in the treatment of childhood tumors?

A

Surgical removal: Most useful
Radiation
Chemotherapy
Multidisciplinary teams

102
Q

Locations of brain tumors

A
103
Q

Neuroblastoma
Is an aggressive tumor. T/F

A

TRUE

104
Q

Originates outside the CNS in the developing sympathetic nervous system

A

Neuroblastoma

105
Q

Causes approximately 50% of all solid tumors in children in the first year of life.

A

neuroblastoma

106
Q

Neuroblastoma causes approximately ___% of cancer deaths in children of all ages.

A

15

107
Q

Where are neuroblastomas usually located?

A

Is usually located in the retroperitoneal area (65%), most often in the adrenal medulla.
Approximately 15% originate in the mediastinum.

108
Q

Cervical neuroblastomas cause _____

A

Horner syndrome
E.g. miosis, ptosis

109
Q

What are s/s of neuroblastoma?

A

Weight loss, irritability, fatigue, and fever
Increased amounts of catecholamines and associated metabolites in urine

110
Q

What 3 diagnostic tools are usued to evaluate a neuroblastoma?

A

Imaging studies
Laboratory studies
Biopsy

111
Q

What are the treatments low, intermediate, high risk tumors and recurring tumors a/w neuroblastomas?

A

Low-risk disease: Surgery
Recurring tumors: Chemotherapy
Intermediate-risk tumors: Surgery and chemotherapy
High-risk tumors: High-dose chemotherapy and radiotherapy, followed by transplantation of purged autologous bone marrow; monoclonal-antibody–based immunotherapy

112
Q

Approximately ____% of children with high-risk disease (neuroblastoma) die, despite intensive therapy.

A

60

Pathopharm Exam 3 (18 decks)

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