Neuromuscular Round 1:
Structural Abnormalities: Remember 80/10/10 Rule
Hydrocephalus
Spina Bifida
Craniosynostosis
Arteriovenous Malformation



Primary diagnostic tests are the CT and MRI with sedation in younger children.

Hydrocephalus:
Impaired CSF absorption
Block in the flow of CSF

Cerebral Spinal Fluid:
Functions: Buffering, Buoyancy, Excretion of waste, Endocrine medium to transport hormones
Total CSF = 125 to 150 ml
Production = 400-500/day (0.36ml/minute)


Etiology/Pathology:
Acquired congenital disorder
Unknown etiology




Communicating hydrocephalus:
Obstruction outside ventricular system causing decreased absorption, most common in premature after a subarachnoid hemorrhage or infection.

Noncommunicating or obstructive hydrocephalus:
Usually obstruction due to a malformation such as aqueductal stenosis, meningomyelocele (MMC), Dandy-Walker syndrome or Arnold-Chiari malformation (brain stem compression due to herniation diff. swallowing, stridor, apnea, respiratory distress, absent gag reflex, upper extremity weakness or spasticity).

Older children or adolescence may develop hydrocephalus from tumors, infection, or head injury

Shunts placed soon after diagnosis have a better prognosis. May have some ataxia, decreased fine motor coordination, developmental delays, spasticity.

Case study Baby Klaus:

Baby Klaus has a severe case of Hydrocephalus. He displays several signs and symptoms, which characterize this condition. Watch the video above and answer the questions.

1. Describe Baby Klaus’s presenting symptoms.
2. What is the name of the condition and what caused it?
3. What is the solution to the problem?
4. What are the nursing concerns (diagnoses) and care (interventions) prior to surgery?
5. What are the nursing concerns (diagnoses) and care (interventions) after surgery?
6. Compare and contrast the symptoms of hydrocephalus in an infant with those of an older child with hydrocephalus.


Ventriculoperitoneal shunt (VP shunt)









Signs and symptoms are related to increased CSF imbalance - 80/10/10 Rule
leading to increased intracranial pressure as the brain is pressed against the interior skull.

Infants - bulging fontanelles, high pitched cry, irritability, lethargy, vomiting, strabismus,
Increasing head circumference, protrusion of the frontal region, translucent skin, wide sutures, visible sclera above eye (sunsetting sign). Positive Macewen's sign - hollow percussion sound, feeding disturbances,

Older children complaining of headaches or visual disturbances, nausea, vomiting, irritability, confusion, lethargy, apathy, reasoning impaired, change in motor abilities.

Treatment directed toward relief, most often surgical, removal of lesion, insertion of shunt. ventriculoperitoneal most common or ventriculoatrial. Shunt malfunction from kink, migrating, plug = IICP

Signs of a shunt infection:
Ventriculitis with low grade fever, malaise, nausea, headache.
Blood cultures may be negative, increase in serum leukocytes
CSF increase in WBCs, protein, decrease in glucose, pathogen seen in cultures or gram stain
Sepsis with modest fever and weakness, positive blood, CSF, leukocytosis and anemia

*Signs of Increased Intracranial Pressure opposite of shock* low heart rate, high blood pressure*

Assessment and nursing care preorperatively:
Head circumference
Signs and symptoms of IICP
Respiratory status
Intake and output
Nutrition - small frequent feeds due to vomiting, may be difficult to hold
Positioning due to large head
Change position and sheepskin to decrease skin breakdown


Assessment and nursing care postoperatively:
Vital signs and neuro checks every two hours
Pain management
Intake and output
Skin integrity
Signs of infection
Observe for IICP
Flat on unoperated side
Vital signs, neuro checks
Observation for abdominal distention and peritonitis due to the irritating nature of CSF
Observe for signs and symptoms of an illeus due to shunt placement, check bowel sounds
Family teaching, lifelong condition, s&s infection, normal lives, no contact sports

Neural Tube Defects
Ancephaly
Cranioschisis
Exencephaly
Encephalocele
Spina bifida occulta
Rachischisis
Spina bifida cystica: Meningocele, Meningomyelocele (hydrocephalus is often a result of this condition)



Preoperative care:
Prone, tactile stimulation, rubbing, stroking
Moist sterile dressing with normal saline
Integrity of sac maintained
Watch for signs and symptoms of infection - IICP, fever, irritability, nuchal rigidity,
Neuro assessment for comparison, extremity movement
Isolette maintain temperature
Skin care
Hip alignment
Feeding challenging head to the side bottle, breast milk pumped

Postoperative care:
Prone position
Antibiotics
Watch infection and hydrocephalus
Bowel, bladder function
Maternal care
Family teaching, s&s infection, hydrocephalus, IICP, urinary/bowel difficulties, shunt failure

Case study Mounib:

Mounib is 2 years old. It is a miracle he is still alive. As stated by the doctors he owes his life to his mother’s expert and conscientious care. Watch the video and answer the following questions.

1. What is the technical name for Mounib’s condition?
2. What are the challenges of this condition prior to repair?
3. What are the post-surgical nursing concerns (diagnoses)?
4. What post-surgical nursing care will this entail (Interventions)?
5. What are possible complications of this condition short and long term?


*Infants, children and adults with spina bifida are 40% more likely to have a latex allergy*

Neuromuscular Round 2
Congenital Neuromuscular Disorders:

Cerebral Palsy

Emma's Cerebral Palsy





Cerebral Palsy**

Cerebral Palsy (CP) is a nonprogressive injury to the motor centers of the brain, which can cause spastic and involuntary movements. CP can also be associated with developmental delays and seizure activity. Persistence of tonic neck reflex and moro reflex beyond 4 months.



Though the exact cause is often unknown, birth injuries, maternal infections, kernicterus, and low birth weight are felt to contribute to this disorder. It can range in presentation from mild to severe.



Diagnosis:
MRI, hx, genetics, EEG, hearing, vision tests useful in diagnosis. Approximately 30-50% mentally disabled with some with cognitive delays. Vocational rehabilitation and higher education possible.



Assessment may reveal:

Poor head control after 3 months
Stiff arms, legs, back arching
Floppy, limp
Cannot sit unsupported after 8 month
Irritability, crying
Feeding difficulties, choking

Classifications:

Spastic – one or both side, hypertonicity, impaired fine motor skills, increased reflexes, scoliosis common, gait scissoring, contractures common, elbows, wrist and fingers flexed.

Dyskinetic/Athetoid – involuntary movements of the face, extremities, involvement of oral muscles causes drooling and speech difficulties, increased movements with stress, not during sleep, normal deep tendon reflexes, contractures are rare.

Ataxic – uncoordinated, unsteady, hyporeflexia, speech slurred jerky, few orthopedic problems.

Mixed – Combination of spastic and dyskinetic

Management:

Early recognition and optimum development
Establish communication, locomotion, self care
Gain optimum appearance of motor functions – ankle foot orthoses (braces) worn to prevent deformity and as an assistive device, scooter devices,
Correct defects as much as possible – corrective surgeries to help contractures or lengthen tendons
Provide appropriate educational opportunities
Promote socialization, promote self image
Pain may occur with spasms, valium, Baclofen < spasms
Botox injected into muscle to < muscle movement
Antiepileptic drugs – Tegretol, Depakote < seizures

Clinical Manifestations:

Neonatal reflexes after 6 months (Moro, tonic neck)
Delayed development
Poor suck, tongue thrust
Spasticity
Scissoring of legs
Involuntary movements
Seizures

Nursing Interventions:

Care coordination: community support agencies, PT, OT, speech therapist, neurologist, orthopedist, nutritionist.
Support and assist with chronic sorrow, grief
Administer anticonvulsants as needed


*Emma’s case study:

Emma has a milder form of cerebral palsy as it is more localized. She does a great job explaining what has caused this and how she feels about it. Watch the video and answer the following questions.

  1. What are typical presenting symptoms of cerebral palsy and suspected causes?
  2. What are common nursing diagnoses and related nursing interventions for cerebral palsy?
  3. What nursing diagnoses and interventions would be specific for Emma?
  4. Is this a genetic condition? Will it worsen with time?
  5. Compare and contrast Cerebral Palsy with SMA and Muscular Dystrophy.


Zachary's Cerebral Palsy



Cerebral Palsy ... Cohen



Zachary, Emma and Cohen demonstrate the differing abilities and appearances of Cerebral Palsy. This is a non-progressive condition resulting from prenatal, perinatal or postnatal central nervous system damage or dysfunction. Though the exact cause is not known the prevalence has increased with the rise in survival rates of pre-term low birth weight and very low birth rate infants.

*Cerebral Palsy Case Study:

Compare and contrast the different types of cerebral palsy depicted in the videos above (Emma, Zachary, Cohen) and the resulting nursing diagnoses and nursing interventions specific to each case.


Spinal Muscular Atrophy (SMA)
Thought to be autosomal recessive genetic disorder
Muscles weaken and waste away (atrophy) due to degeneration of motor neurones (nerve cells in the spinal cord).
Motor neurons relay signals from the brain to the muscle cells.
When motor neurons fail to function, the muscles deteriorate.
Brain and the sensory nerves (that allow us to feel sensations such as touch, temperature, pain etc.) are not affected.
Intelligence is normal.


Three common types of SMA in childhood:
SMA Type 1 Severe -Werdnig-Hoffman Disease
SMA Type 2 Intermediate
SMA Type 3 Milder - Kugelberg-Welander Disease

The major difference between these types are the age of onset and the severity of the condition.



Infantile Spinal Muscular Atrophy, SMA Type 1, (Werdnig-Hoffman Disease)
Inherited autosomal-recessive
Genetic analysis, EMG, muscle biopsy
Affects anterior horn cells of the spinal cord producing muscle atrophy
Most severe form of SMA
Evident in the first six months of life.
The child is unable to roll or sit unsupported
Severe muscle weakness eventually causes feeding and breathing problems
These children usually do not live beyond about 18 months of age.
Have normal intelligence
Verbal, tactile and auditory stimulation important
Often succumb to respiratory problems

Juvenile Spinal Muscular Atrophy, SMA Type 3, (Kugelberg-Welander disease)
onset after 2 years of age
milder than the infantile or intermediate forms





*Case’s case study:

Case’s video is a story of love and sacrifice. These parents are so devoted to their child; it is touching and inspiring.

  1. Knowing what we know about the different types of SMA, what type does Case probably have?
  2. What is his prognosis?
  3. He seems cognitively developmentally appropriate; is this what we would expect?
  4. Name the main nursing diagnoses for Case and the related nursing care. His parents have pretty much provided this for you.
  5. Compare and contrast SMA and Muscular Dystrophy.


Muscular Dystrophy

Largest muscular disease in children
Inherited disease
Degeneration of muscle fibers, wasting of muscles
Genetic analysis, EMG, muscle biopsy

Facioscapulohumeral

Face and shoulder muscles
Autosomal dominant
Appears in teen years

Limb-girdle

Autosomal recessive
Late childhood/adolescence
Pelvic and shoulder muscles

Duchenne (Pseudohypertrophic)

Duchenne most common x-linked recessive, males
Appears ages 3-5
Progressive
Respiratory and cardiac complications
Often fatal by age 25

Manifestations:

Waddling gait
Clumsiness
Gower's sign difficulty rising
pseudohypertrophy of muscles (fat deposits)
Muscle degeneration/weakening
Cognitive delays
Scoliosis, respiratory and cardiac difficulties > wheelchair

Nursing Interventions:

Assistive services
Coordinate PT, OT, neurologist, orthopedist, nutritionist.
Protect from exposure to illness
Active and passive exercise
Dietary support to decrease obesity
Support, chronic sorrow, grief



Duchenne Muscular Dystrophy Timeline



The emotional side of DMD, Ian's Story



***Case Study Comparisons:

The Duchenne Muscular Dystrophy (DMD) Timeline is very telling as it notes the changes, which occur over time with Muscular Dystrophy patients. Ian’s story demonstrates the rollercoaster a family goes through with chronic sorrow.

  1. Note the various physical changes that occur over time with DMD patients.
  2. What are some of the top nursing concerns (nursing diagnoses) and some of the resulting nursing interventions (nursing care) for the child and family of a child with DMD?

Acquired Neuromuscular disorders

Lead Toxicity
Screened at age 1 and 2
Cleanse the site, finger stick
If positive repeated with a venous draw
Can effect renal, hematological and neurological
Ingested because it is sweet, sometimes cultural, dust, in soil, toys, pipes
Handwashing, wash toys, do not eat from pottery, drink cold water

Rarely have symptoms
Can cause encephalopathy and cerebral edema
Anemia

Treatment depending on level
<10 reassess in 1 year
Environmental investigation, management 20-44
Chelating agents > 45




Guillain-Barre Syndrome
Postinfectious Polyneuritis
Uncommon, acute, demyelinating polyneuropathy ascending
Children < adults
Most common 4-10
Immune mediated associated with illness and vaccines
3 phases:
Acute may last 4 weeks with progressive symptoms
Plateau symptoms remain the same, can last for days or weeks
Recovery pt begins to improve, from days to weeks

Symptoms:
Muscle tenderness
Parasthesia
Muscle weakness
Ascending
Reflexes absent
Incontinence
Recovery is descending, with legs last

Diagnosis:

CSF has increased protein
EMG shows symmetric denervation versus polio

Treatment:

Supportive
May need respiratory and pharyngeal support
IV immunoglobulin
Steroids
Plasmapheresis
Immunosuppressive drugs

Nursing Considerations:
Supportive
Observe for respiratory depression, difficulty swallowing
Vital signs and neuro checks
Recovery, prevent complications
Communication
ROM, PT






Tetanus
Lockjaw
Preventable, can be fatal

Causative organism:
anaerobic spore forming gram + bacillus Clostridium tetani
Occurs in a susceptible host after injury of tissue and introduction of spore
Spore in intestines of humans, animals and soil

Signs and Symptoms:

Stiffness and tenderness of muscles in neck and jaw
Difficulty opening mouth
Sardonic smile, muscle spasm
Board like rigidity of abdomen and limbs
Sensitivity to stimuli causes muscle spasms
Laryngospasm
Tetany of respiratory muscles
Patient anxious and alert

Nursing Consideration:

Decrease stimulation
Low light, low sound
Observe respiratory status
Emergency equipment
Muscle relaxants, opiods, sedatives
Monitor O2 saturation
IV hydration
Nutrition
Suctioning as needed
Due to awareness it is terrifying
Comfort, explain and do not leave alone





Botulism
Flaccid Paralysis due to anaerobic bacillus Clostridium botulinnum
Toxin in contaminated food source
Often in Honey and corn syrup, so none till over 12 months old
Wound botulism
Infant botulism
Man made botulism

Diagnosis:
History,
Physical
Blood and stool cultures
EMG

Treatment:
IV administration of botulism antitoxin
Supportive measures
Recovery may take 44 days
Death rate 2%

Activities:

*Name ways to prevent lead poisoning and why this is important.

*Sofi’s Case Study:

Briefly describe the progression and recovery of Guillain-Barre Syndrome. Are there any treatments?

***Compare and contrast Tetanus and Botulism.


Spinal Cord Injuries







References:

Axton, S. E., & Fugate, T. (2003). Chapter 8: Care of Children with musculoskeletal dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 187-216). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc.

Axton, S. E., & Fugate, T. (2003). Chapter 9: Care of children with neurological/neuromuscular dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 217-252). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc.


Videos:
Cerebral Palsy
https://youtu.be/cOfUGUNxEqU

Emma speaks about CP
http://youtu.be/1dxRYY_qbP0

Zachary's Cerebral Palsy
http://youtu.be/u0kTAPDCo7A

Cohen's Story
http://youtu.be/Kzb1XYGO0IQ


A day in the life of SMA
http://youtu.be/Vj6TZdEzXj0

BrainPop Animation about Duchenne Muscular Dystrophy
http://youtu.be/6wLnR7GJakY

Duchenne Muscular Dystrophy Timeline
http://youtu.be/KA8W5UfE4ts

Ian's Story MD
http://youtu.be/cY3M_A_BkG0

Videos:

Lead Poisoning
http://youtu.be/hNUcN7yf1cc

Guillain-Barre syndrome
http://youtu.be/Zwr83v96y2M

Tetanus
http://youtu.be/AzCDKC06WLw
References:

Axton, S. E., & Fugate, T. (2003). Chapter 8: Care of Children with musculoskeletal dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 187-216). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc..

Axton, S. E., & Fugate, T. (2003). Chapter 9: Care of children with neurological/neuromuscular dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 217-252). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc..

Videos:
Spina Bifida
https://youtu.be/YG4nrRnAlcc

Hope for baby Klaus
http://youtu.be/WkWguVEx-uk

Insertion of a CSF shunt
http://youtu.be/Qmym2iFVNw8

Shunt for hydrocephalus
http://youtu.be/0h7Xa-Lsnac

Spina Bifida Surgery Mayo Clinic
http://youtu.be/E080qJuHWdQ