Altered Neurological Functions

1. Altered Neurological Functions Najwa Subuh- MSN in Pediatric

2. Assessment of cerebral functions • Young children < 2 years require special evaluation because they are unable to responds directions • Early neurologic responses are primarily reflexive, replaced by meaningful movement. This evidence of progressive maturation reflects more extensive myelinization & changes in neurochemical & electrophysiological properties Najwa Subuh- MSN in Pediatric

3. Most information about infants & small children is gained through observation of elicited reflex responses, development of increasingly complex locomotors & fine motor skill, & eliciting progressively communicative & adaptive behaviors, delay or deviation from expected milestones • Obtain history of the pregnancy, delivery, respiratory status at birth, neonatal health to determine the effect of intra uterine & extra uterine environmental influences that affect the maturation of the CNS Najwa Subuh- MSN in Pediatric

4. History • The history should carefully document in chronological order the onset of symptoms & a thorough description of their frequency, duration, & associated characteristics. • Most children beyond the age of 3–4 yr are capable of contributing to their history, particularly about facts relating to the present illness. Najwa Subuh- MSN in Pediatric

5. It is essential to obtain a comprehensive review of the function & interaction of all organ systems, because abnormalities of the CNS may initially present with clinical manifestations (e.g., vomiting, pain, constipation, or UT disorders). • A detailed history might suggest that the child's vomiting is due to increased (ICP), that the pain behind the eye may be caused by migraine headaches or multiple sclerosis, & that the constipation & urinary dribbling may be due to a spinal cord tumor. Najwa Subuh- MSN in Pediatric

6. Physical Examination • Size & shape of the head • Activity, postural reflex activity, sensory responses • Extremities movements • Facial features; high pitch cry • Hyperventilation, abnormal respiratory cycle, prolonged apnea • Level of development, neurologic functions • Muscular activity & coordination including ocular movements & gait, facial movement & mouth functions Najwa Subuh- MSN in Pediatric

7. Assess LOC by using motor cues • Test attention span & concentration by asking the child to repeat a series of numbers after you • Test the child's recent memory by showing him a familiar objects, waiting 5 minutes, the asking him to recall the object Najwa Subuh- MSN in Pediatric

8. Developmental delay; the nurse' role is to establish a baseline measure, support the child & family to assist in the provision of services • Often children with developmental delays have scattered performance, exhibiting one age of development in the physical domain, another in the language domain & another in the personal-social or self-help domain • This assessment includes a thorough history, which focus on risks factors, description of gait & achievement of developmental milestones. Najwa Subuh- MSN in Pediatric

9. Cerebral Palsy • CP; is a non-progressive disorder accompanied by perceptual problems, language deficits & intellectual involvement Najwa Subuh- MSN in Pediatric

10. Spastic Dyskinetic/athetoid Ataxic dystonic Najwa Subuh- MSN in Pediatric

11. Clinical classification Najwa Subuh- MSN in Pediatric • Spastic; may involve one/both sides “hemiparesis/quadriparesis” • Hypertonicty with poor control of posture, balance • Impairment in fine & gross motor • It represents an upper motor neuron type of muscular weakness increased stretch reflexes, increased muscle tone, hypotonia or decreased tone Dyskinetic/athetoid Abnormal involuntary movement, slow wormlike, writhing movement involve the extremities, trunk, neck, facial muscle & tongue Affect pharyngeal, laryngeal & oral muscles  drooling & dysarthria “imperfect speech articulation” Jerky movement, dystonic in muscle tone

12. Ataxic • Wide-based gait • Rapid, repetitive poorly performed movement • Disintegration of movement of upper extremities when the child reaches for objects • Mixed type/ dystonic; combination of spasticity & athetoid Najwa Subuh- MSN in Pediatric

13. Causes • Prenatal • Genetic syndrome • Chromosomal abnormalities • Brain malformations • Intrauterine infection • Perinatal • Sepsis, CN infection • Asphyxia • Prematurity • Labor & delivery • Preclampsia • Complicated labor • Hyperbilirubinemia. Hemolytic disorders. • Respiratory distress. Infections. • Electrolyte disturbances (hypoglycemia, hypocalcemia). • SGA • Cerebral trauma during delivery. • Childhood • Meningitis • Traumatic brain injury • Toxins • Vascular accidents. • Anoxia. • Neoplastic & late neuro-developmental defects. Najwa Subuh- MSN in Pediatric

14. Pathophysiology • No pathogenic picture, but there are gross malformations of the brain vascular occlusion, atrophy, loss of neurons • Anoxia plays the most significant role in the pathologic state of brain damage • CP associated with prematurity is usually spastic diplegia caused by hypoxic infarction or hemorrhage in the lateral ventricle • In athetoid type of CP caused by kernicterus & hemolytic disease of the newborn, pigment deposits in the basal ganglia & some cranial nerves nuclei. • Cerebral hypoplasia, neonatal hypoglycemia are related to ataxic • CP; generalized cortical & cerebral atrophy  severe quadriparesis with mental retardation & microcephaly. Najwa Subuh- MSN in Pediatric

15. Complications • Impaired physical mobility • Self-care deficits • Physical injury • Impaired communication • Mental impairment • Contracture Najwa Subuh- MSN in Pediatric

16. Clinicalmanifestations • All types • Delayed gross motor development • Abnormal motor performance & coordination; can manifest early in life as poor sucking & feeding difficulty • Posture abnormality occurring at rest or when changing position • Altered muscle tone • Increased or decreased resistance to passive ROM • Opisthotonic postures "exaggerated arching of the back" • Spasticity of hip muscle & lower extremities, making diapering difficult Najwa Subuh- MSN in Pediatric

17. Abnormal reflexes • Persistent primitive reflexes • Other disabilities • Mental retardation of varying degrees in 18% - 50% of patients "most children have at least a normal IQ but can't demonstrate it on standardization tests" • Seizures • ADHD: distractibility, deficit of integration • Sensory deficits –vision-hearing- speech Najwa Subuh- MSN in Pediatric

18. Drooling; Contribute to wet clothing & skin irritation, abnormal posture & motor performance, alteration in muscle tone, affect chewing, swallowing & talking • Aspiration; Coughing & chocking especially while eating • Inadequate gas exchange; uncoordinated & weak respiratory efforts • Orthopedic complications; Unilateral or bilateral hip dislocation, scoliosis, joint contracture due to unbalanced muscle tone • Constipation; Due to decreased mobility & difficulty in toileting, difficulty in eating bulky foods because of uncoordinated chewing & swallowing Najwa Subuh- MSN in Pediatric

19. Dental caries; It results from: • Improper dental hygiene • Congenital enamel defects “hypoplasia of primary teeth” • High carbohydrate intake & retention • Dietary imbalance with proper nutritional intake • Inadequate fluoride • Difficulty in mouth closure & drooling • Oral hypersensitivity  resists dental hygiene Najwa Subuh- MSN in Pediatric

20. Therapeutic Management • Goal of therapy: • Early recognition & promotion of an optimum development course • Therapy is symptomatic & preventive only disease is permanent • Early recognition & diagnosis provide the sensorimotor experiences for cognitive development • Establish locomotion, communication & self help • Gain optimum appearance & integration of motor function • Correct associated defects • Provide educational opportunities adapted to the needs & capabilities of the individual child • Promote socialization experiences with other affected & unaffected child Najwa Subuh- MSN in Pediatric

21. Delivers baclofen, a skeletal muscle relaxant, directly to the intrathecal space around the spinal cord • Use to treat spasticity • Pump last for 3-5 years, after that time, a new pump must be implemented • Muscle relaxant or neurosurgery to decrease spasticity • Anticonvulsant “phenytoin & luminal” to control seizures • An artificial urinary sphincter may be indicated for the incontinent child who an use the hand control • Orthopedic surgery to correct contractures • Braces or splints & special appliance such as adapted eating utensils & a low toilet seats with arms, to help the child perform activity independently Najwa Subuh- MSN in Pediatric

22. Nursing Diagnoses • Impaired physical mobility related to altered neuromuscular functioning • Delayed G&D related to the nature & extent of the disorder • Interrupted family processes related to the nature of the defect, the demands of daily management, and resultant changes in family life • Risk for Injury related to deficit in motor activity and coordination Najwa Subuh- MSN in Pediatric

23. Nursing Interventions • Institute a high- calorie diet for the child with increased motor function to help him up with increased metabolic demands • Perform ROM exercises to minimize contracture • Assist with locomotion, communication & educational opportunities • Promote age appropriate mental activities & incentives for motor development to promote G&D • Make food easy to manage to decrease stress during meal time Najwa Subuh- MSN in Pediatric

24. Provide rest period to promote rest & reduce metabolic needs • Provide a safe environment for example, have the child use protective headgear or bed pads to prevent injury • Provide tasks into small steps to promote self care & activity & increase self esteem • Refer the child for speech, nutrition & physical therapy to maintain or improve functioning • If the child can’t speech, use assistive communication devices to promote a positive self concept • Assist family members in setting realistic goals & managing stress Najwa Subuh- MSN in Pediatric

25. Mental retardation; significant sub-average general intellectual functioning existing concurrently with deficits in adaptive behaviour & manifested during the developmental period • Adaptive behaviours; maturation, learning skill & social adaptation of the person. Najwa Subuh- MSN in Pediatric

26. Causes of mental retardation • Prenatal factors; genetic defects, chromosomal abnormalities, complex malformation syndrome, toxic exposure, congenital defects, Rh, ABO incompatibility, toxemia, placental insufficiency or Antepartum hemorrhage, infections • Perinatal factors; complications with prematurity, hypoxic-ischemic episode, infection, maternal overdose of medication during labor • Post natal factors; childhood diseases, accidents, anoxia infection e.g. meningitis,, poisoning, Hyperbilirubinemia, influences in the child’s environment, metabolic disorder, trauma, severe deprivation Najwa Subuh- MSN in Pediatric

27. Diagnosis:Delay in language, cognitive skills, gross motor skills Classification of mental retardation • Mild retarded; (educable IQ range 55-69) • Individuals may be able to learn academic skills to sixth grade level & are able to master simple occupational skills if given opportunities & instructions • Social & communication skills are good, they may be able to help support themselves as adults • Moderately retarded; (treatable IQ range 40-54) • Persons can learn the basics of self care in childhood & functional academically at the low grade level • They may be able to accomplish simple work with very close supervision Najwa Subuh- MSN in Pediatric

28. Severely retarded (IQ range 25-35) • People need to a controlled environment in which by adulthood, they are able to learn the skills of communication, self-protection, hygienic & sheltered/workshop vocation • They may learn a few wards & have basic communication skills • Profound retarded (IQ range < 25) • People usually need complete care & supervision during all their lives, but may show some motor & speech development • They have very limited self-care skills Najwa Subuh- MSN in Pediatric

29. Down Syndrome “DS” • Etiology: • Unknown • Genetic predisposition; 3-6% of the cases = translocation of chromosome 21 • Radiation prior to conception • Infection • Advanced maternal age: age of 35 = risk is 1/385 Age of 40 = risk is 1/106 Age of 45 = risk is 1/30 Najwa Subuh- MSN in Pediatric

30. Abnormal physiological functioning affects thyroid metabolism “hypothyroidism” & intestinal malabsorption, frequent infection due to impaired response, • Decreased buffering of metabolic processes results in predisposition to hyperuricemia & increased insulin resistance, DM develops cataract, Alzheimer disease, bone marrow dysfunction is indicated by leukemia. Najwa Subuh- MSN in Pediatric

31. Clinical manifestations • Intelligence • Severely retarded – low average intelligence • Social development • 2-3 years beyond the mental age especially during early childhood • Sensory problems • Ocular problems = strabismus, nystagmus, myopia, cataract • Up slanting palpebral fissures Najwa Subuh- MSN in Pediatric

32. Premature aging, early graying or loss of hair, ↓skin tone • Mouth & teeth, tongue protrusion, fissured & furrowed tongue, mouth breathing, drooling, malformed teeth • Nose, Hypoplastic nasal bone, flat nasal bridge • Chest; internipple distance is decreased, short rib cage • Abdomen; umbilical hernia, protruding • GI; duodenal atresia, Hirschsprung disease, imperforated anus • Skeleton; short & broad hands, muscle weakness, hyper-extensible finger joints, hypotonic Najwa Subuh- MSN in Pediatric

33. Growth • short stature & obesity • Ht, Whg is reduced ↑ whg • Behaviour; warmth, cheerful, gentleness, patience • Psychiatric disorders; autism, ADHD, obsessive compulsive disorders Najwa Subuh- MSN in Pediatric

34. Complications • Seizures disorders, 5-10% tonic-clonic seizures • Delayed Growth • Physical injury • Aspiration • Death • Hearing loss, conductive or mixed or sensorineural losses, OM, narrow canal, impacted cerumen Najwa Subuh- MSN in Pediatric

35. Sexual development • Delayed incomplete or both • Women with DS are fertile • Men with DS are infertile • Congenital anomalies • Congenital heart disease “40-50%” endocardial cushion defect, VSD, ASD, TOF, PDA • Renal agencies • Duodenal atresia, Hirschsprung disease, TEF • Skeletal defect • Microcephaly Najwa Subuh- MSN in Pediatric

36. Therapeutic Management • “No cure for DS” • Surgery; to correct serious congenital anomalies • The presence of DS alone doesn’t adversely affect the outcome of surgery in the absence of pulmonary hypertension • Adeno-tonsillectomies for obstructed sleep apnea • Prevent dental caries through appropriate dental hygiene, fluoride treatment, good dietary habits Najwa Subuh- MSN in Pediatric

37. Early intervention program • Feeding, fine & gross motor development. Early gavage feeding if necessary because the infant’s sucking reflex may be poor • Language; personal & social development • Evaluation of sight & hearing • Treatment of OM • Special growth chart Najwa Subuh- MSN in Pediatric

38. Atlantaxial instability; symptoms include neck pain, weakness, however most affected children are asymptomatic. • Screening done on the 2nd birthday & before engage in physically active exercise or sports • If children become symptomatic, they should receive prompt attention because risk of spinal cord compression Najwa Subuh- MSN in Pediatric

39. Genetic counseling; recurrence risk = 1% • Immunization & medications • Usual immunization • Test for thyroid hormones to prevent intellectual deterioration • Sub acute bacterial endocarditis prophylaxis • Digital & diuretics for cardiac mgt • Treat skin disorders with whg reduction, frequent bath Najwa Subuh- MSN in Pediatric

40. Nursing Considerations • Provide activities & toys appropriate for the child to support optimal development • Support family at time of diagnosis • Promote child’s developmental progress • The hypotonicity affects muscular development, supporting skills may be delayed • Stimulation programs is encouraged • Developmental screening tests to evaluate indications of progress e.g. strength, balance, coordination or muscle tone • Investigate appropriate day care programs Najwa Subuh- MSN in Pediatric

41. Assist family in preventing physical problems • The extended body position promotes heat loss, because more surface area is exposed to the environment • Parents are encouraged to swaddle or wrap the infant tightly in a blanket to provide security & warmth • Discuss with parents about feeling & concerns of attachment Najwa Subuh- MSN in Pediatric

42. Decreased muscle tone compromises respiratory expansion under developed nasal bone  chronic problem of inadequate drainage of mucus  URTI, ear infections, so clearing the nose, increased fluid intake, change the child’s position, performing postural drainage • Feeding, large protruding tongue & hypotonic interferes with feeding, tongue thrust is a physiologic response not a cause for feeding refusal • Dietary intake needs supervision, ↓ muscle tone affects gastric motility  constipation • Careful monitoring to prevent obesity Najwa Subuh- MSN in Pediatric

43. Assist in prenatal diagnosis & genetic counseling • Provide activities & toys appropriate for the child to support optimal development • Set realistic reachable goal break tasks into small steps to make them easier to accomplish • Use behavior modification, if applicable to promote safety & prevent injury to the child & others • Provide stimulation & communicate at a level appropriate to the child's mental age rather than chronological age to promote a healthy emotional environment Najwa Subuh- MSN in Pediatric

44. Provide a safe environment to prevent injury • Mainstream daily routines to promote normalcy • Encourage parents to care for, bond with & hold their child • Teach parents to perform all of the above interventions because care will mostly be provided at home by the parents Najwa Subuh- MSN in Pediatric

45. Head Injury Causes: • Motor vehicle related accidents • Child abuse • Vigorous shaking • Bicycle accidents especially in those without helmets • Sports accidents especially in those without helmets • Falls Najwa Subuh- MSN in Pediatric

46. Pathophysiology • The intracranial components are damaged because of a force too great to be absorbed by the skull, muscles, & ligaments that support the heads • The skulls of infants & children are pliable & can absorb much of the physical impact, providing some level of protection to the intracranial components, but they have a larger head size & less support from muscle & ligaments making them more prone to acceleration – deceleration injuries Najwa Subuh- MSN in Pediatric

47. Types of head injury • Scalp laceration- can cause a child to bleed to death because of the vascularity of the surface area • Epidural, intracranial hemorrhage- bleeding into the space between the dura mater & the skull • Subdural hemorrhage- bleeding between the dura mater & the archnoid layer of the meninges • Concussion- a transient state of neurologic dysfunction caused by a jarring of the brain Najwa Subuh- MSN in Pediatric

48. Contusion- sign of petechial hemorrhage on the superficial aspects of the brain at the site of the impact • Skull fracture • Linear –simple • Depressed- depression of a bone towards the brain • Basilar- "at the skull base" Najwa Subuh- MSN in Pediatric

49. Complications • Hemorrhage • Infection • Edema • Herniation Najwa Subuh- MSN in Pediatric

50. Assessment findings • Change in LOC or mental status • Confusion • Restlessness • Irritability • Pale skin • Vomiting • Increased head circumferences • Bulging fontanels • Hemiparesis, quadriplegia • Headache • Decreased memory • Diminished pupillary responses Najwa Subuh- MSN in Pediatric