1. Neurological conditions and diseases Post Basic Paediatrics 18 April 2012
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. Neurological conditions and diseases Part I At birth (Congenital, acquired)
4. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
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. Macrocephaly: Causes SCALP caput succedaneum, subgaleal hemorrhage, and cephalohematoma
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. 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. 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. 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. 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. 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. Macrocephaly: Causes BRAIN PARENCHYMA -megalencephaly Parenchymal space enlargement occurs in neurocutaneous disorders, Soto syndrome, metabolic megalencephalies, and some degenerative disorders. Brain Tumors
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. 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. 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. 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. Hydrocephalus: Causes Acquired - Post meningitis - Post hemorrhage- (SAH, IVH) - Masses - vascular malformations, neoplastic
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. 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. 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
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. 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. 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. 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. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
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. 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. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
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. 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
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. Meningocele Posterior vertebral arches fail to fuse Sac-like protrusion containing meninges and cerebral spinal fluid No spinal nerve involvement
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. 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. 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. 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. 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. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
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. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE
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. 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. Mental Retardation Significant below average intellectual functioning which is associated with impaired learning difficulties Causes Pre-natal Perinatal Post-natal
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. 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. 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. 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. Interventions Goal is to promote Optimal development Family support Community referrals
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. 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. 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. Treatment Self-care is a goal for all children Team approach Nutrition Increased caloric intake Special feeding devices Community referrals Emotional support
58. At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIE