Family Exam 4 (Chapter 49)

What are the 2 key characteristic features and prevalence of cerebral palsy in childhood?

1. It is characterized by abnormal muscle tone and coordination.

2. It is the most common permanent disability in childhood

What co-morbidity do children with Cerebral Palsy often have?

Epilepsy.

What 9 increased risk factors are associated with cerebral palsy?

1. Perinatal infections

2. Pre-term birth

3. Perinatal ischemic stroke

4. Bacterial meningitis

5. Viral encephalitis

6. MVCs

7. Child abuse

8. Periventricular leukomalacia

9. Intracerebral hemorrhage

What plays the most significant role in the pathologic state of brain damage in cerebral palsy, and what are the 4 types of movement disorders?

1. Anoxia plays the most significant role.

2. 4 types of movement disorders

   1. Spastic

   2. Dyskinetic

   3. Ataxic

   4. Mixed

What are the 5 components of the clinical classifications for cerebral palsy?

1. Motor abnormalities

2. Associated impairments

3. Anatomic & radiologic findings

4. Causation

5. Timing

What are the 6 clinical manifestations of cerebral palsy?

1. Delayed gross motor development

2. Abnormal motor performance

3. Alterations in muscle tone

4. Abnormal posture

5. Reflex abnormalities

6. Associated disabilities and problems

What are the 4 ways cerebral palsy is diagnosed?

1. Infants at high risk require careful assessment in early infancy.

2. Primary: neurological exam and history

3. Neuroimaging

4. Metabolic and genetic testing

What are the 5 primary goals of therapeutic management for cerebral palsy?

1. Establish locomotion, communication, and self-help skills.

2. Optimal appearance and integration of motor functions

3. Correct associated defects as effectively as possible

4. Educational opportunities

5. Promote socialization

What 4 management strategies are utilized in the therapeutic care of cerebral palsy?

1. Mobilization devices

2. Surgery

3. Medication

4. Technical aids

What are some associated problems with cerebral palsy and who deals with this?

Physical and speech issues; occupational therapists and SLP help.

What is true of fragility and life span in children with cerebral palsy?

More medically fragile = less likely to survive into adulthood.

What has caused a decline in the prevalence of spina bifida?

Food fortification with folic acid and supplementation recommendations.

When does spina bifida occur during fetal development, and approximately when does the neural tube close?

1. Occurs when the neural tube (structure that forms brain and spinal cord) doesn’t close.

2. Closes 1 month after conception.

What are the 5 multi-factorial origins or causes associated with neural tube defects like spina bifida?

1. Drugs

2. Radiation

3. Maternal malnutrition

4. Chemicals

5. Genetic mutations

What is myelodysplasia?

Any malformation of the spinal canal and cord.

What is spina bifida, and what are its two main types?

1. Spina bifida is the most common defect of the CNS. Midline defects involving the failure of the osseous spine to close.

2. 2 main types → occulta, cystica

How do spina bifida occulta and spina bifida cystica differ in appearance?

1. Occulta → not visible externally

2. Cystica → visible defect with an external saclike protrusion

What 5 neuromuscular disturbances usually accompany the defects of spina bifida?

1. Progressive/static changes in gait

2. Foot weakness

3. Foot deformity

4. Partial paralysis of the lower extremities

5. Bowel and bladder sphincter disturbances

What are the 2 major forms of spina bifida?

1. Meningocele

2. Myelomeningocele

What is a meningocele, and does it involve neural elements or neurological defects?

1. A meningocele is a type of spina bifida that involves a membranous sac of spinal fluid that pushes through a gap in the spine.

2. It has NO neural elements.

3. It has NO neurological defects.

What is a myelomeningocele, and during what timeframe of pregnancy does it develop?

1. A myelomeningocele is a sac containing the meninges, spinal fluid, and part of the spinal cord that pushes through the gap in the spine protruding out of the back.

2. It develops during the first 28 days of pregnancy.

How and where is myelomeningocele typically located?

1. Detected prenatally or at birth.

2. Common in the lumbar or lumbosacral area

What determines the extent of neurological impairment with myelomeningocele?

The location (PARALYSIS BELOW LUMBAR).

What 2 other conditions are associated with myelomeningocele?

1. Hydrocephalus

2. Chiari Malformation Type II

What are the 4 manifestations of myelomeningocele?

1. Depend on the degree of the defect

2. Bladder defects

3. Anal sphincter tone

4. Joint deformities

What is anencephaly in relation to neural tube defects, and what are its key structural characteristics?

1. It is the MOST SERIOUS neural tube defect

2. Both cerebral hemispheres are absent and the brain protrudes through the skull.

What is the prognosis for anencephaly, and what type of care is provided?

1. It is incompatible with life (live several hours to weeks).

2. Comfort measures and palliative care is provided

What team approach and timeframe for surgical closure are indicated in the nursing management of neural tube defects?

1. Multidisciplinary approach

2. Surgical closure within 24-72 hours

What are the 4 key infection and trauma prevention measures for neural tube defects?

1. Prevent infections

2. Prevent trauma to the site due to risk of rupture

3. Prevent drying of the sac

4. Change dressings frequently

What 4 ongoing assessments and positioning requirements are necessary for the infant with neural tube defects?

1. Perform neurological assessments.

2. Monitor output (myelomeningocele is one of the most common causes of neurogenic bladder)

3. Place the infant in an incubator to regulate temperature.

4. Keep the infants in a prone position

Why might diapering be a concern, and what other special considerations are highlighted in neural tube defects?

1. Diapering may be contraindicated.

2. Avoid LATEX exposure

What 4 foods are people with latex allergies at risk for eating?

1. Avocado

2. Banana

3. Chestnut

4. Kiwi

What is the inheritance pattern of Werdnig-Hoffman disease?

Autosomal recessive.

What type of syndrome is Werdnig-Hoffman disease most commonly associated with?

Floppy infant syndrome / congenital hypotonia

What happens to skeletal muscle in Werdnig-Hoffman disease?

It progressively weakens and wastes.

When is the typical age of onset of Werdnig-Hoffman Disease?

Variable but usually before 2 years.

What posture and 3 physical signs are typical for an infant with Werdnig-Hoffman Disease?

1. Frog position

2. Weak cry

3. Cough

4. Generalized muscle weakness

Where is active movement usually limited to in Werdnig-Hoffman Disease?

Finger and toes.

Are sensation and intellect affected in Werdnig-Hoffman disease?

No.

What is the usual prognosis and primary vulnerability of Werdnig-Hoffman Disease?

1. Death usually by 2 years old.

2. The primary vulnerability is respiratory infections.

What is Guillain-Barré Syndrome (GBS)?

Infectious, polyneuritis, an uncommon acute demyelinating polyneuropathy with progressive paralysis.

What is the hallmark sign of GBS?

Acute peripheral motor weakness.

When does paralysis usually occur in relation to triggers in GBS?

Paralysis occurs approximately 10 days after an acute/nonspecific viral infection/administration of certain vaccines.

At what age range does GBS most often occur in children, and how are children affected compared to adults?

1. Occurs most often in ages 4-10.

2. It is not as common in children as opposed to adults.

What type of disease process is GBS, and what causes nerve impairment?

It is an immune-mediated disease. Nerve impairment is caused by edema in spinal and cranial nerves. This impairs nerve conduction.

What prodromal symptoms typically precede the paralysis of GBS?

A mild flu-like illness/sore throat.

How does onset and muscle weakness progress in GBS?

Rapid onset, peaks within 24 hours or a gradual progression over days/weeks.

What neurological sensations accompany the muscle tenderness in GBS?

Paresthesia and cramps.

What is used for diagnostic evaluation and treatment of GBS?

1. Spinal tap for diagnosis.

2. Treated with I.V. IgG and steroids

What is the typical recovery timeline and 2 nursing care priorities for GBS?

1. Recovery begins within 2-3 weeks, most regain full strength

2. Prevent contractures (via ROM exercises)

3. MONITOR AIRWAY

What is another name for tetanus?

Lockjaw.

What organism causes tetanus?

Clostridium tetani.

What type of bacterium is C. tetani?

Anaerobic, spore forming, and gram-positive.

What does C. tetani produce that causes disease?

An exotoxin.

Where are the 3 places C. tetani spores found?

1. Soil

2. Dust

3. GI tract of humans/animals

How can the bacteria enter the body in tetanus?

A scratch, wound, burn, or crushed area.

Which muscles are affected first by tetanus?

The masseter and neck muscles. They present with rigidty.

What is the incubation period for tetanus?

3 days to 3 weeks.

What are the 3 early signs of tetanus?

1. Progressive stiffness and tenderness of the neck and jaw.

2. Difficulty opening the mouth.

3. Facial muscle spasms.

What are the 5 progressive signs of tetanus?

1. Opisthotonos

2. Difficulty swallowing

3. Laryngospasms

4. Tetany of respiratory muscles

5. Rigidity of the abdomen and limbs

What is opisthotonos?

Rigid, extreme backward arching of the head, neck, and spine caused by intense, involuntary muscle spasms

What are the 3 main ways to prevent tetanus?

1. Immunization and boosters

2. Prophylactic therapy

3. Good wound care

What are the 3 conditions for tetatnus?

1. Presence of tetanus spores or vegetative forms of bacillus.

2. Injury to the tissues.

3. Wound conditions that encourage multiplication of the organism.

4. 
   

In what activities is risk for exposure to tetanus greater?

Outdoor activities.

What is botulism?

Serious food poisoning resulting from ingestion of the preformed toxin produced by anaerobic Clostridium botulinum.

What are common sources of botulism?

Improperly sterilized home canned foods.

Which foods should never be given to infants because of botulism risk?

Honey and corn syrup.

When do CNS symptoms appear after ingestion in botulism?

Abruptly, 12-36 hours after ingestion.

What are the 5 main clinical signs of botulism?

1. Weakness

2. Dizziness

3. Headaches

4. Speech issues

5. Vomiting

What is the life-threatening complication of botulism?

Respiratory paralysis.

What are the 4 signs of infant botulism?

1. Weak cry

2. Constipation

3. Breathing problems

4. Excessive drooling

What is the main treatment for botulism?

IV botulism immune globulin.

What 2 supportive measures are provided for botulism?

Respiratory and nutritional support.

How long is therapy continued in botulism?

Until paralysis subsides.

What is the most severe and most common muscular dysfunction of childhood?

Duchenne muscular dystrophy (DMD).

How is DMD inherited?

X-linked recessive (typically new mutations).

At what age does DMD typically begin?

3 and 7 years of age.

What are the 3 hallmark features of DMD?

1. Progressive muscle weakness

2. Wasting

3. Contractures

What happens to the calf muscles in DMD?

Hypertrophy.

Relentless progression of DMD leads to death from what?

Respiratory or cardiac failure.

What is Gower sign in DMD?

Childs walk using their hands to stand up from a squatted position on the floor.

What gait changes are seen in DMD?

A waddling gait with frequent falls.

What spinal change is seen in DMD?

Lordosis.

What happens to the muscles in later stages of DMD?

They atrophy.

What are other manifestations of DMD?

Mild to moderate mental impairment, obesity.

Is there an effective treatment for DMD?

No. Treatment is supportive.

How do you help maintain function in DMD?

Keep the child as active as possible and maintain function of unaffected muscles.

What 4 therapies are used to manage contractures in DMD?

1. ROM exercises

2. Bracing

3. ADLs

4. Surgical release if needed

What medications may be given for DMD?

Prednisone or corticosteroids.

What support should the family receive in DMD?

Genetic counseling.