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

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. 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

23. Hydrocephalus: Assessment
 Complications:
- Shunt blockages
- Infection of shunt
- Over shunting
- Seizures
- Blindness
- Cranial nerve dysfunction
- ICP
- Cognitive impairment

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

32. Neural Tube Defects
 Spina bifida
 Diastematomyelia

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

35. Spina Bifida
 Occulta
 Meningocele
 Myelomeningocele

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

59. Thank You