Neural Tube Defects (NTD)

• Development, Cognition, and Mobility, Week 7

Neural Tube Defects (NTD)

• NTD is when the neural tube fails to close completely during embryonic development.
• Neural tube is the structure in a developing embryo that becomes the infant's brain, spinal cord, and surrounding tissues.
• Neural tube folds and closes early in pregnancy, around weeks 3-4.
• Failure to close can occur anywhere along the brain, neck, or spine.
• NTD types include Anencephaly and Spina Bifida.
• Spina Bifida includes Spina Bifida Occulta, Meningocele, and Myelomeningocele.
• Etiology factors for NTD include folic acid deficiency, previous NTD pregnancy, maternal malnutrition, exposure to drugs/radiation/chemicals, and genetic mutation.
• Daily folic acid intake for childbearing women is 0.4mg.
• NTD causes and risk factors are not always known but are associated w/ factors interfering with CNS development such as folate deficiency, malnutrition, and maternal obesity
• Other causes and risk factors include Infections, Medications (Sodium Valproate & Methotrexate) and exposure to Chemicals.

Neural Tube Defects (NTD): Anencephaly

• It is a congenital malformation where both cerebral hemispheres are absent.
• Most infants are stillborn or die shortly after birth
• Infants are born with a portion of the brainstem.
• They can maintain temperature, cardiac, and respiratory function for hours to weeks, but will eventually die of respiratory failure.
• There is no specific treatment.
• Clinical manifestations include the infant born without a forebrain or cerebrum with remaining brain tissue exposed.
• The condition is incompatible with life, with most infants dying in the womb or within hours to weeks after birth.
• Background includes a fatal birth defect where the infant is born without most of the brain and overlying skull/scalp, resulting in death shortly after birth.
• Risk factors include genetics, environment, geography, nutrition, Hispanic ethnicity, female sex at birth, hot tub use during pregnancy, lack of folic acid, and use of anti-seizure medications.
• Signs & Symptoms
• Newborns born without parts of brain tissue, skull, and scalp. The superior part of the head is typically absent or particularly small.
• Some brain tissue may be exposed
• Resulting in the baby being unconscious, blind, deaf, and without pain sensitivity.
• If part of the brainstem evolves, reflexes like breathing or responding to touch/sound may be possible.
• Absent parts of the brain responsible for swallowing could lead to Polyhydraminos.
• Possible Fetal malpositioning, placental abruption, and premature birth.
• Diagnosis is made through ultrasound, elevated alpha-fetoprotein in maternal serum, and physical examination.
• There is no cure or treatment, but prevention is done to reduce incidence through early detection, diagnosis, and folic acid supplement.

Neural Tube Defects (NTD): Spina Bifida

• Spina Bifida Cystica types include of Meningocele and Myelomeningocele.
• Meningocele: the sac contains spinal fluid and meninges but no spinal contents, may or may not have neurological defects.
• Myelomeningocele: the sac contains spinal fluid, meninges, and nerve with decreased motor and sensory function depending on lesion location.
• In both Spina Bifida Cystica types the cyst can be seen visible as a cyst of the meninges through the Vertebrae.
• Myelomeningocele: the spinal cord herniates out of the vertebral defect, making it easier to damage
• Increasing the Risk of developing motor and sensory dysfunction below the defect.
• Possibility of meningitis, hemorrhage, and hypoxia
• Causing Bladder/ Bowel Incontinence
• Spina Bifida Occulta
• Frequently has no observable manifestations, but can be associated with cutaneous manifestations like skin depression/dimple, port-wine stain, dark tufts of hair, and soft, subcutaneous lipomas
• May have progressive neuromuscular disturbances such as gait problems and bowel/bladder difficulties.
• Spina Bifida Cystica symptoms depend on the anatomic level of the defect and nerves involved
• Leading to Bowel and bladder difficulties, flaccid extremities, and joint deformities from in utero positioning.
• Also causing Varied degrees of sensory deficits
• Hydrocephalus often present
• Spinal defects
• Spina Bifida Occulta:
• Posterior vertebral arches fail to close, but the spinal cord and meninges remain in place.
• The defect is not visible from the outside.
• Spina Bifida Cystica defects:
• Parts of the spinal cord herniate through an abnormal gap in the vertebrae, forming a cyst.
• Meningocele involves only meninges herniating into the cyst.
• Myelomeningocele contains meninges and spinal cord.
• Other associated conditions
• Latex allergy; repeated exposure during surgery and procedures
• Tethered spinal cord: the spinal cord attaches to the spinal canal and stretches as the child grows, creating spinal nerve damage.
• Paralysis and mobility limitations which vary depending on type and severity of the spina bifida.
• Hydrocephalus, seen most frequently in myelomeningoceles.
• Care for Newborns W/ Myelomeningocele priority goals: Psychological support, Prevent Complications, and minimize Heat & Fluid loss.
• Myelomeningocele Surgical Repair:
• Fetoscopic repair is a surgical repair that occurs while the fetus is still in utero
• Traditional repair is on baby right after birth
• Dressing changes post-op to prevent sac rupture, should consist of sterile, moist, non-adherent dressings.
• Ensuring the dressing should not dry out and Remaining in prone position prior/post surgery.
• Infant should be fed with head turned to side with NG placement while being monitored wit Axillary temps.
• Prevent skin breakdown with Gentle ROM, checking the head circumference.
• Spina Bifida is diagnosed prenatally with Amniotic Fluid & Maternal Blood test for AFP & Acetylcholinesterase.
• After Birth can be diagnosed with Physical Examination, Imaging, Cranial Ultrasound to asses for Hydrocephalus.
• This defect can be prevented wit Folic Acid Supplementations.
  • Ideally through Cesarean Section
• priority nursing goals include minimizing heat & fluid loss, preventing complications and psychosocial suport.
• Client and Family Teaching shoul include how to care of the baby at home when to seek medical attention and how to prevent future NTDs in future pregnancies

Cerebral Palsy

• CP is a non-progressive, but not unchanging, neuromuscular disorder with posture and movement limitations from static brain injury prenatally or postnatally.
• Abnormal brain development or damage occurs to the cerebral cortex, basal ganglia, or cerebellum.
• Etiology, clinical features, and course vary but are characterized by abnormal muscle tone and coordination, often impacting sensation, perception, communication, cognition, and behavior.
• Many experience secondary musculoskeletal problems and epilepsy.
• Signs and symptoms include delayed gross motor development, abnormal motor performance, altered muscle tone and posture, reflex abnormalities, and visual/hearing/speech/intellectual problems or seizures.

Cerebral Palsy Risk Factors

• Prenatal risk factors: maternal diabetes/hyperthyroidism/infections/bleeding, gestational congenital malformations, fetal development abnormality, placental issues, and teratogens.
• Labor & Delivery risk factors: Premature delivery, Prolonged rupture of membranes, prolonged fetal heart rate depression, long labor, preeclampsia, Asphyxia, or abnormal presentation.
• Perinatal risk factors: sepsis/CNS infection, seizures, intraventricular hemorrhage, meconium aspiration, intrauterine growth restriction, low birth weight, or perinatal stroke.
• Childhood/Postnatal Risk Factors include Brain injury, Meningitis / Encephalitis, Toxins, Traumatic brain injury, Infections , and Stroke.
• Unknown prenatal factors can contribute to 70-80% of CP cases.
• Cerebral Palsy types:
• Spastic (Pyramidal): tense, contracted muscles with classifications into monoplegia, hemiplegia, diplegia, or quadriplegia.
• Dyskinetic: Constant, uncontrolled motion of limbs/head/eyes that interferes with feeding, swallowing, and speech.
• Ataxic: Poor sense of balance causing falls/stumbles with wide-based, unsteady gait.
• Mixed Type: Combination of spastic and dyskinetic CP.

Cerebral Palsy Complications and Nursing Considerations

• Complications includes, Aspiration because patients cant handle Drooling.
  • Nurse can prepare action, keeping bed Elevated and Parents know CPR.
• Potential for Injury, Patient should be place on padded rails
• Nurse should be aware of seizure precautions and Encourage rest.
• Goals of Therapy incude, Early Recognition and enabling the children to attain potential.
• Nursing Care:
• Repositioning patient with Frequent skin assessment.
• When feeding, place put food to side of mouth
• The Nurse Should use Jaw control to assist with mouth closing by gentle pressure under chin.
• Seizure precautions and also Fall prevention should be take , cooridinating Home/School care
• Medications for this include; Baclofen (muscle spasticity), Diazepam (should be restricted to older children/adolescents), and Botulinum toxin A (used primarily for clients who only have spasticity in LE, monitor for temporary weakness and pain at injection site. Anti-epileptics

Prematurity

• Preterm: infant is born before 37 weeks of gestation.
• Late preterm: infant is born between 34 0/7 and 36 6/7 completed weeks of pregnancy.
• Common Conditions: Apnea, Respiratory distress syndrome, Retinopathy, Jaundice, Hemorrhage, Infection, Sepsis & Anemia.
• Maternal risk factors: previous premature birth, pregnancy with multiples, less than 6 months between pregnancies, IVF, uterus/cervix/placenta problems, smoking, illicit drug use, infections (amniotic fluid and lower genital tract), HTN/diabetes, under/over weight before pregnancy, stressful life events, miscarriages/abortions, trauma.
• Signs and symptoms: extended limbs, poorly developed ear cartilage, fine/feathery hair, lots of wrinkles on the sole of the foot and scarf sign.
• Apnea of Prematurity (AOP): "Apneic spells" are common in preterm babies, less than 32 weeks.
  • Apnea is a spontaneous breathing lapse for >20 seconds or brief pauses alongside bradycardia or oxygen desaturations.
  • It is attributed to undeveloped respiratory reflexes and muscle weakness.
  • The Infection worsens apneic spells
  • Often resolved around 37 weeks
• Management involves caffeine, closed monitoring and tactile stimulation of the baby.
• Retinopathy of Prematurity (ROP): primarily affects premature infants and can result in blindness. The more premature an infant is, the greater the ROP risk.
• High oxygen levels increase ROP risk in infants.
• Sepsis refers to generalized bacterial infection in the bloodstream, which preterm infants are highly susceptible to due to an immune system that still has not been fully developed.
• The IgG is normally acquired from the maternal system and stored in the fetus in the final weeks of gestation. -Early clinical manifestations are nonspecific, and can Hypothermia , Changes in color, tone, activity, feeding behavior, Sudden episodes of apnea and Unexplained oxygen desaturation events.
• Prevention should be aimed to be preformed using Hand hygiene and Breastfeeding
• General Care for Preemies:
• Minimize sensory stimuli by Decreasing sound, lights and using Clustering cares → after 30 weeks gestation
• Pain and stress reduction to avoid Increased sensitivity to pain. Frequent pain can lead to lifelong structural and functional Alterations in nervous systems/ pain responses
• Positioning similar to intrauterine environment with extremity flexion and hip adduction while containing the Neonate promotes healthy growing.
• Often improves vital sign, Breathing and deeper sleep while In prone position

Kangaroo Care

• A method of holding newborn skin to skin Stabilizing the infant's heart rate, Reducing illness and Infection, Enhancing breastfeeding, Improving breathing pattern/oxygen saturation levels, Better sleep, Decreased crying, and increased bonding.

Intracranial Regulation

• Balance of 3 components within the skull: brain tissue (80%), cerebrospinal fluid (CSF) (10%), and blood (10%).
• If any component's volume increases, another must decrease to maintain normal intracranial pressure (ICP).
• The body can compensate for volume changes within the skull by altering CSF production/absorption, increasing/decreasing cerebral blood flow, and brain tissue compensation.
• If volume continues to rise despite compensation, ICP will increase.
• Increased ICP decreases blood flow to the brain, leading to compression and ischemia.

Hydrocephalus

• A condition caused by an imbalance in the production and /or absorption of CSF.
  • CSF accumulates in ventricles of brain becoming dialated.
• Can be Congenital or Acquired Conditions
• Congenital conditions causes include: Myelomeningocele, Aqueduct stenosis and Intrauterine viral infections such as Cytomegalovirus (CMV) and Toxoplasmosis.
• Acquired includes: Intraventricular hemorrhage, Tumor, CSF infection, or Head Injury
• Communicating Hydrocephalus (Non-obstructive) - No Obstruction the flow within the Ventricular System
  • Impaired absorption or increased amount of CSF.
• Noncommunicating Hydrocephalus.
  • Obstruction the flow within the Ventricular System.
• Clinical Manifestation will Vary between infants and older children/Adults:
• Infants will show symptoms such as Bulging Fontanels, Setting Sunsign Eyes and a Rapid increase in head circumference
  • May also contain High Pitched cry, becomes Irritable and Lethargic
• Adults & Older childred Will experience Headaches on Awakening, Nausea and Vomiting, difficulties with walking and Poor Memory.
  • May also complain of, vision change or Change in LOC (Difficulties concentrating)
  • If you can't resolve pressure then leads to Somnolence, Seizures, Cardiopulmonary distress , and/or Brain death.
• Therapeutic Management should be directed at relieving Ventricular pressure.
  • Should be addressed by treating the underlying cause and any complication causing problems.
  • And finally, Treatment can be by way of Surgically removing obstructions using, a Tumor , or Cysts.
    • Also, Can be treated with Shunt procedures like Ventriculoperitoneal (VP) or Ventriculoarterial (VA) Shunt.

Hydrocephalus VP Shunt Complications

• Infection being the most common w/in 2 Months.
  • Can be treated w/ IV intra-thecal antibiotics
• Recurrent infections may require removing shunt
  • this can be temporary
• A malfunction leading to Kinking, Plugging, tubing migration or the beginning of Thrombus formation at the distal end of tubing.
• Nursing Patient Management to Monitor Hydrocephalus
• Place paint on un-operated side with Patient remaining flat.
• devices/magnets at minimum of 3cm from shunt valve, Usually behind ear!
• Perform Neuro and abdominal assessment
• Check Vitals signs and perform Daily head circumference monitoring!
• Provide Patient, Family support, educating on what to look or that would signal a dysfunction or infection.

Mobility

• Purposeful physical movement, including gross motor, fine motor, and coordination, which needs:
• Intact/functional neurological and musculoskeletal systems, and anatomical function for a change in position or location that demonstrates purposeful, pain-free movement.
• This enhances quality of life through improved functional ability, joint mobility, muscle strength, and system functioning (cardiac, respiratory, GI). Risk factors: older adults, chronic pain, injury/trauma, and congenital deformities.
• Negative consequences includes Reduction in muscle mass, atrophy, joint contraction, pressure ulcer development, anxiety, boredom or a Disturbed the body image.

Parkinson's Disease

• A chronic, progressive neurodegenerative disorder with Characteristics such as, Bradykinesia, Rigidity, Blank facial expression, slow monotone speech , and Tremor while resting.
• Etiology remains to be unknown, but may include Well Water.
• Risk factors are degeneration of dopamine producing neurons in the brain, and results in imbalance of dopamine and acetylcholine in the basal ganglia.
• The main Diagnostics is by patients history and having, Presence of TRAP with asymmetric onset!

Parkinson's Disease Collaborative care

• Nurse should be aware of side effects such as: spontaneous involuntary movement, Hallucinations, Dementia, Depression, Dysphagia and Aspiration or injuries such as Falls.
• Medications that could support collaborative care are: Levodopa/Carbidopa which Corrects imbalance of neurotransmitters, Benztropin (Cogentin) to help Decreases muscle stiffness/saliva and Selegiline anti-depressent.
  • Also big point is, Don't give Levodopa with food! patient should consult pt, ot/st, Dietician for best treatment.

Strokes

• Ischemic stroke: obstruction from a clot that is blocking blood flow to the brain.
• Hemorrhagic stroke: blood vessel ruptures which is stopping blood flow to the brain.
• Transient ischemic attack (TIA): caused by a temporary clot.
• Strokes are classified based on the underlying pathophysiologic findings: ischemia from inadequate blood flow or hemorrhage from bleeding.
• Loss of function varies according to brain tissue's location and extent of involvement.
• Prevalence: Currently 5th leading cause of death in US and Leading cause of serious, long-term disability.
• Survivors: 15-30% will experience permanent disability, requiring longterm care for both the survivor and family.
• Risk factors: include Nonmodifiable (Age, Gender , ethnicity, Family History) and/or Modifiable (Hypertension, Heart Disease, Diabetes, Tobacco Use, and Obesity.
• Can be identified in it's in early recognition using the, (B)E FAST or (F)AST acronym

Strokes Transient Ischemic Attack

• Transient episodes of neurologic dysfunction caused by focal brain, spinal cord or Retinal Ischemia, but without acute infarction of the brain.
  • this is, Associated with an increased RISK
• symptoms typically last less than 1 hour. 1/3rd will progress to an ischemic stroke!
• Thrombotic being most common (60 %)
  • Its devlopes where Atherosclerosis plaques have already causes narrowing
• Embolic Occurs when an embolus occludes a cerebral artery
• Most emboli originate in the endocardial layer of the heart Can also be sudden onset
• Patient will typically be put on a course of, Treatment aTPA (clot buster) Anti-Coagulation therapy.
  • It's common too that Males > Women
• It's commin to that, TIA typically makes this Uncommon and it is on Sudden onset, activity!
• Males > W, ~1/3 (30%) experience TIA but During or after sleep.
• Prevention of Strokes- (THIS IS KEY!)
• Diet High Fruites and Veggies, consider Diet in case its duto Clog, prevent formation of Atherosclerosis by lowering fat and NA+ levels!
• NO Tabacco and Alcohol intake and Reduce Salt and/or Sugar in your Meals/Foods
• Take as Many meds prescribed!
• During a Stroke, it important to Assess respiratory status
• You provide position and, Asses presence of gag reflex! Assess Manifestation of Stroke in, infusing TPA for patients who will meet criteria
• This is Important because it needs to be, Administer within 3 hrs!
• Continue by monitoring Cardiovascular & neurologic status Giving Repostion/ROM.

Hemorrhagic Stroke

• Intracerebral stroke from bleeding within the brain caused by a rupture:
  • Possible cuases Hypertension, coagulation disorders, anticoagulant therapy, or ruptured aneurysms.
  • Patient will also experence frequent Headaches
• Subarachnoid : Intracranial bleeding into the CSF space between the arachnoid and pia meter
• this can be caused by Ruptured Aneurysm, Trauma and Illicit Drug use.
• Patient will more then often have Sudden onset
• Can be treated using: - Surgical decompression if indicated - Clipping or coiling of aneurysm - Tight-lacing
  • The chances for Hemorrhaging are More Common In Females
• For a Stroke what is importantis to always: THINK FAST
• Hemorrhagic stroke

Right & Left Brain Damage

• Right-Brain Damage

  • Paralysis or weakness occurs on the Left's side of the body and results in being, hemiplegic.

• You also must take in to account, the Lefts, Spatial neglect.

  • It takes more work to solve spatial-perception problems. And It may also have an Impulsive nature.
    • In contrast also on other side is a decrease in attention on short attention spans when trying to perfoam a new actions

• Left-Brain Damage-

  • Paralysis or weakness occurs on the (R) s/o the body resulting in being, hemiplegic.

• Difficulty with speech occurs, typically in verbal-expressive language

  • Slow or hesitant in language and a decreased attention span.

• Diagnostic Studies:

• Can be perform to check the Extents of the damage with CT. Also MRI and Cerebral and Carotid angiography

• A CBC helps check Lipid profiel For Therapies- PT, Speech and OT, you most know!

• Families can give support but also are impacted because a Family Member would need assist and maybe relocated