Neurological Conditions and Diseases (At birth)


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Neurological Conditions and Diseases (At birth)

  1. 1. Neurological conditions and diseases Post Basic Paediatrics 18 April 2012
  2. 2. Neurological conditions and diseases Part I  At birth (Congenital, acquired)  Macrocephaly  Microcephaly  Spine defect  Other developmental defect  Birth trauma/HIE Part II  During development (Congenital, acquired)  Meningitis  Seizure  Headache  Stroke/Vascular  Neoplasm/Tumour  Trauma  Coma
  3. 3. Neurological conditions and diseases Part I At birth (Congenital, acquired)
  4. 4. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  5. 5. Macrocephaly Macrocephaly during the neonatal period results from enlargement of any component or “space” of the head The components or spaces of the head most likely to enlarge are the scalp, skull, subdural space, subarachnoid space, brain parenchyma, intraparenchymal vessels, and ventricles.
  6. 6. Macrocephaly: Causes SCALP  caput succedaneum,  subgaleal hemorrhage, and  cephalohematoma
  7. 7. Caput Succedaneum Caput succedaneum is due to edema between the skin and the epicranial aponeurosis. It presents as a mass, usually located in the vertex, that crosses the sutures and extends over several bones. The mass is soft, superficial, and pitting. The edema results from compression of the scalp by the uterus or suction on the scalp if a vacuum extractor was used during delivery
  8. 8. Subgaleal Hemorrhage Subgaleal hemorrhage is due to blood between the epicranial aponeurosis and the external periosteum. Subgaleal hemorrhage presents as an evenly spread mass throughout a large portion of the scalp. The mass is firm, fluctuant, crosses suture lines, and increases in size after birth (sometimes at an alarming speed)
  9. 9. Cephalohematoma Cephalohematoma presents as a localized mass that does not cross suture lines. It is usually unilateral and over the parietal bone. The blood collects between the external periosteum and the bone. The mass is firm, tense, and confined to an individual bone. The edge of the mass may feel like a ridge. Underlying linear fracture is detected in 10% to 25% of cases. Cephalohematoma is produced by forces that tend to separate the periosteum from the bone.
  10. 10. Macrocephaly: Causes SKULL -Osteopetrosis  Osteopetrosis is a disorder characterized by overgrowth of brittle bones.  This results in thick, dense, and fragile bones.  The bony tissue overgrowth results in encroachments of the:  (1) bone marrow leading to anemia,  (2) cranial nerves foramina leading to deafness, blindness, or other signs of cranial nerve dysfunction,  (3) Pacchioni bodies producing communicating hydrocephalus and macrocephaly.
  11. 11. Macrocephaly: Causes SUBDURAL SPACE -Subdural hematomas  Progressive increases in head circumference may be noted during the third week of life.  Subdural hematomas present with irritability or hyperalertness, or with signs of focal cerebral disturbances such as seizures, hemiparesis, or gaze preference.  The causes of subdural hematomas are trauma and coagulation disorders.  Subdural hematoma is diagnosed by CT of the brain.
  12. 12. Macrocephaly: Causes SUBARACHNOID SPACE  Patients with benign enlargement of the subarachnoid space are usually not born macrocephalic;  However, some patients with this condition may have excessive head growth during the neonatal period.  The presence of bilateral enlarged frontal subarachnoid spaces (>5.7 mm), widening of the Sylvian fissure (>7.6 mm) and other sulci, and normal or minimally enlarged ventricles establishes the diagnosis.  The anterior fontanelle is large and soft to palpation.  Family members, most often the father, may also have a large head
  13. 13. Macrocephaly: Causes BRAIN PARENCHYMA -megalencephaly  Parenchymal space enlargement occurs in  neurocutaneous disorders,  Soto syndrome,  metabolic megalencephalies, and  some degenerative disorders.  Brain Tumors
  14. 14. Macrocephaly: Causes Vein of Galen Aneurysm  Neonates with aneurysm of the vein of Galen may be macrocephalic at birth.  The most common neonatal presentations of vein of Galen aneurysm in the neonatal period are cardiac failure, cerebral infarction, or cerebral bleed.  Macrocephaly can be caused by the large size of the vein of Galen aneurysm, but most often it is caused by an obstruction of the aqueduct of Sylvius.  A cranial bruit is often present in neonates with vein of Galen aneurysm.
  15. 15. Macrocephaly: Causes Hydrocephalus   Increased amount of CSF within the ventricles of the brain  May be caused by obstruction of CSF flow or by overproduction or inadequate reabsorption of CSF  May result from congenital malformation or be secondary to injury, infection, or tumor
  16. 16. Hydrocephalus Types:  Communicating hydrocephalus: - Results from unsatisfactory absorption of CSF by the arachnoid gratulations or overproduction of CSF by the choroid plexus  Non-communicating hydrocephalus: - Results from an obstruction to CSF flow , causing enlargement of only those ventricles proximal to the obstruction
  17. 17. Hydrocephalus: Causes Congenital  Aqueductal anomalies - Primary aqueductal stenosis, or secondary to intrauterine infections i.e. varicella, mumps, TORCH - Dandy-Walker malformation - Chiari malformation - Myelomeningocele
  18. 18. Hydrocephalus: Causes Acquired - Post meningitis - Post hemorrhage- (SAH, IVH) - Masses - vascular malformations, neoplastic
  19. 19. Clinical Presentation Increased head circumference- Irritability, lethargy, poor feeding, vomiting -infant- Headache, lethargy, vomiting- older child- Bulging anterior fontanelle- Widened cranial sutures- Cracked pot sound on cranial percussion- Scalp vein dilatation
  20. 20. Clinical Presentation Sunset sign - eyes deviate downward- Episodic bradycardia, apnea- Loss of color and peripheral vision(older child)- Cranial nerve palsies - e.g abnormal pupil size/reactivity, EOM’s, nystagmus- Spasticity limbs- Hyperreflexia, clonus
  21. 21. Hydrocephalus: Assessment Assessment findings depend on    age of onset and  amount of CSF in the brain Infant to 2 years:  Enlarging head size, bulging, non-pulsating fontanels, downward rotation of eyes   (sunset), poor feeding, vomiting, lethargy, irritability, high-pitched cry   and abnormal muscle tone Older Children:  Changes in head size less common  Signs of increased ICP (vomiting, ataxia, headache) common  Alteration in consciousness and papilloedema late signs
  22. 22. Hydrocephalus: Assessment Diagnostic Investigations:  Ultrasound of skull- through anterior fontanelle  Shows ventricular enlargement  CT of head - Shows ventricular enlargement, peri-ventricualr lucency, narrow/absent sulci, +/- 4 th ventricular enlargement Treatments:  Serial Spinal taps  Surgery- remove obstruction if possible  Shunts  Acetazolamide- decreases blood flow to choroidal arteries , therefore decreasing CSF production
  23. 23. Hydrocephalus: Assessment Complications: - Shunt blockages - Infection of shunt - Over shunting - Seizures - Blindness - Cranial nerve dysfunction - ICP - Cognitive impairment
  24. 24. Shunts Insertion of a flexible tube into the lateral ventricle of the brain Catheter is the threaded under the skin and the distal end positioned in the peritoneum (common) or the right atrium Shunt drains excess CSF from the lateral ventricles; fluid is the absorbed by the peritoneum or absorbed in the general circulation via the right atrium
  25. 25. Shunts : Nursing Interventions Pre-operative  Monitor head circumference  Monitor for signs of ICP  Small frequent feedings Post-operative  Position on opposite side of surgery or back  Avoid sedation  Monitor for signs of ICP  Educate parents concerning signs and symptoms of shunt infection or shunt malfunction
  26. 26. Macrocephaly: Causes Posthemorrhagic Hydrocephalus  Posthemorrhagic hydrocephalus is the most common type of hydrocephalus in the neonatal period.  Posthemorrhagic hydrocephalus may be communicating or noncommunicating.  It is usually the consequence of intraventricular hemorrhage.  Intraventricular hemorrhage usually occurs as a consequence of germinal matrix hemorrhage.  Germinal matrix hemorrhages are unusual after 34 weeks gestational age.
  27. 27. Macrocephaly: Causes Posthemorrhagic Hydrocephalus  Germinal matrix hemorrhages are classified based on brain ultrasound in four grades.  Grade I intraventricular hemorrhage refers to the presence of subependymal bleed;  Grade II intraventricular hemorrhage refers to extension of the subependymal bleed into the ventricles but without ventricular dilatation;  Grade III intraventricular hemorrhage refers to subependymal bleed with extension of the bleed into the ventricles and hydrocephalus; and  Grade IV intraventricular hemorrhage refers to subependymal bleed with extension of the bleed into the parenchyma as a result of venous infarcts
  28. 28. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  29. 29. Microcephaly Causes include: - Premature closure of skull sutures (craniosynostosis) - Microencephaly - small brain due to insult ( infectious, toxic, metabolic, vascular) sustained in the perinatal or early infancy period e.g rubella,CMV, Fetal alcohol syndrome, Genetic disorder - microencephaly vera, many syndromes and metabolic disorders
  30. 30. Anencephaly Defective closure of the rostral neural tube results in anencephaly or encephalocele Neonates with anencephaly have a rudimentary brainstem, or midrain , no cortex or cranium Rapidly fatal condition if born alive
  31. 31. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  32. 32. Neural Tube Defects Spina bifida Diastematomyelia
  33. 33. Spina Bifida (myelodysplasia) Neural tube defects that develop during the first trimester of fetal development Defect can occur at any place along the spinal canal Unknown etiology; thought to be associated with folic acid deficiency in mother’s diet prenatally Degree of disability dependent on location of the defect & if spinal nerves involved
  34. 34. Spina Bifida (myelodysplasia) Defective closure of the caudal end of NT at the end of 4th week of gestation Results in anomalies of the lumbar and sacral vertebrae or spinal cord Range of severity of CNS defect Preventable with pre-conceptual Folic acid supplements 0.4 mg /day
  35. 35. Spina Bifida Occulta Meningocele Myelomeningocele
  36. 36. Spina bifida “Occulta" Spina bifida "occulta" (meaning "hidden" in latin) Posterior vertebral arches fail to fuse No herniation of meninges or spinal cord May have a tuft of hair or dimpling over the lumbarsacral area No loss of function
  37. 37. Meningocele Posterior vertebral arches fail to fuse Sac-like protrusion containing meninges and cerebral spinal fluid No spinal nerve involvement
  38. 38. Myelomeningocele Sac-like herniation containing meninges, CSF, and spinal nerves imbedded in the wall of the sac There may be no signs or symptoms The spinal arch has not closed, but the spinal cord underneath has retained its normal position and is not damaged Skin of back intact, small dimple or tuft of hair may be present over affected vertebrae A child could grow up and never know that he or she has the defect
  39. 39. Nursing Care – Spina Bifida Neurological status Assess degree of sensation at or below lesion Leg movement Neurogenic bladder Measure head circumference High risk of hydrocephalus High risk for infection High risk for impaired skin integrity Altered urinary elimination Bowel incontinence/constipation Impaired physical mobility
  40. 40. Nursing Care – Spina Bifida Sac  Monitor for leakage of spinal fluid  Monitor skin integrity of sac  Assess for infection- Sac or systemic  Position infant on side or abdomen  Apply wet, sterile, saline dressing  Do not allow sac to dry out
  41. 41. Nursing Care – Post-operative Defect/sac is surgically closed within 48 hours Observe for latex allergies Neurogenic bladder: straight catheterization Neurogenic bowel: bowel management program Monitor for signs/ symptoms of hydrocephalus
  42. 42. Diastematomyelia- A bone or fibrous band divides spinal cord in two longitudinal sections- Associated lipoma may be present, which tethers cord to vertebra- Signs and symptoms include weakness, numbness in feet, urinary incontinence, decreased or absent reflexes in feet- Treatment - surgery to free cord
  43. 43. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  44. 44. Encephalocele Skull defect with exposure of meninges alone or meninges and brain Sometimes defect can cause protrusion of frontal lobe through the nose
  45. 45. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  46. 46. Hypoxic-ischemic Encephalopathy  Hypoxic ischemic encephalopathy (HIE) refers to the CNS dysfunction associated with perinatal asphyxia. HIE is of foremost concern in an asphyxiated neonate because of its potential to cause serious long-term neuromotor sequelae among survivors. A simple and practical classification of HIE by severity of manifestations provided by Levene
  47. 47. Hypoxic-ischemic Encephalopathy  Hypoxic-ischemic encephalopathy often involves the brain and the brainstem. Very severe hypoxic-ischemic encephalopathy may involve the brain, brainstem, spinal cord, and muscle. Magnetic resonance imaging of the brain in neonates with hypotonia due to hypoxic-ischemic encephalopathy shows loss of gray-white matter interface, cortical necrosis, or neuronal loss of the basal ganglia and thalamus. 
  48. 48. Mental Retardation Significant below average intellectual functioning which is associated with impaired learning difficulties Causes  Pre-natal  Perinatal  Post-natal
  49. 49. Mental Retardation: Causes• Pre-natal -Genetic Disorders • Chromosomal aberrations- e.g. Down syndrome (trisomy 21 ) • Disorders with autosomal-dominant inheritance- e.g. Tuberous sclerosis • Disorders with autosomal-recessive inheritance- metabolic disorder; e.g. Phenylketonuria • X-linked mental retardation- Fragile X syndrome • Maternal infections- e.g. Rubella infection during the first month of pregnancy • Toxic substances- fetal alcohol syndrome • Toxemia of pregnancy and placental insufficiency
  50. 50. Mental Retardation: Causes• Perinatal (This period refers to 1 week before birth to 4 weeks after birth ) • Infections -e.g. herpes simplex type 2 • Delivery problems – e.g. birth asphyxia • Other perinatal problems • Retinopathy of prematurity • Hyperbilirubinemia
  51. 51. Mental Retardation: Causes• Postnatal • Infections • Bacterial and viral infections of the brain during childhood may cause meningitis and encephalitis and result in permanent damage • Toxic substances –e.g. Lead poisoning • Other postnatal causes • Childhood malignancies, brain tumors • Trauma • Psychosocial problems –e.g. Severe maternal mental illness • Unknown causes • no cause can be identified in approximately 30% of cases of severe mental retardation and in 50% of cases of mild mental retardation
  52. 52. MR – Classifications Mild  Slow learner, can work, marry, have children, may need assistance with crisis Moderate  Needs life supervision Severe  Needs a caretaker for basic needs Profound
  53. 53. Interventions Goal is to promote  Optimal development  Family support  Community referrals
  54. 54. Cerebral Palsy A non-progressive motor disorder of the CNS resulting in alteration in movement and posture Cause is trauma, hemorrhage, anoxia or infection before, during or after birth 1/3 of children have some degree of mental retardation Classified as:  Spastic  Spasticity (hypertonicity of muscle groups)  Athetoid  Worm-like movements of   extremities  Ataxic  Disturbed coordination  Mixed
  55. 55. Cerebral Palsy – Assessment May have hypertonicity or hypotonia of varying degrees on different extremities May have scissoring of the legs Absence of expected reflexes or presence of reflexes that extend beyond expected age Failure to meet developmental milestones Difficulty swallowing Altered speech
  56. 56. Nursing Care Impaired physical mobility Self-care deficit Altered nutrition: less than body requirements High risk for injury related to neuromuscular, perceptual or cognitive impairments
  57. 57. Treatment Self-care is a goal for all children Team approach Nutrition Increased caloric intake Special feeding devices Community referrals Emotional support
  58. 58. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
  59. 59. Thank You