Case 4

1. Case 4 Group 4

2. General Data

3. Patrick, 2 year old male • From Cavite • Referred from General Pediatrics Clinic for developmental delay General Data

4. History

5. Born at 43 weeks AOG via SVD (spontaneous vaginal delivery) • 29-year old mother G1P1 (1001) • Lying-in clinic in Cavite • Regular prenatal checkups • At the health center • 3 prenatal UTZ: all normal • (+) excessive vomiting (3rd & 4th months) • (-) history of fever, bleeding, hypertension history

6. Inadequate contractions during labor • Born limp, cyanotic, meconium-stained rushed to a tertiary hospital • At the tertiary hospital: • Intubated, admitted at ICU (for 2 weeks) • Diagnosis: pneumonia • Stayed for another 2 weeks • After 3 days (1 month of age): cough and difficulty of breathing  brought to PGH history

7. PGH: • Intubated for 2 days • Treated for pneumonia • After 7 days: discharged • After 1 week (1.5 months of age): readmitted for pneumonia • 3 months of age: • Poor head control • Closed anterior fontanel • Microcephaly history

8. Subsequent months: • Head lag • Absence of regard • Episode of jerking • Stiffening of extremities (esp. when agitated) • Milestones • 1 year: vocalization • 14 months: Spontaneous smile • 15 months: Appear to listen to sound • 18 months: persistence of stiffening diagnosed as epilepsy history

9. Respiratory infection: at least once a month • Dyshidrotic eczema: diagnosed at 1 yr. of age other medical problems

10. (-) similar neurodevelopmental illness • (+) Asthma: mother and materal aunt • (+) HPN: maternal grandmother familyhistory

11. Father • 29 years old • Security guard • High school graduate • Mother • 30 years old • Housewife • High school graduate • Brother • 8 years old • Grade 3 student personal/social history

12. 0-3 months: breastfed • Then gvenBonna at 1:2 dilution • (+) regurgitation of milk • 6 months: given cereal  thrusted out  ricewater (“am”) + milk instead • Currently on Bonamil + am • 6oz. Nine times/day • Slightly thickened solution nutrition history

13. 0-3 months: breastfed • Then gvenBonna at 1:2 dilution • (+) regurgitation of milk • 6 months: given cereal  thrusted out  ricewater (“am”) + milk instead • Currently on Bonamil + am • 6oz. Nine times/day • Slightly thickened solution nutrition history

14. Physical Exam

15. physical exam

16. physical exam

17. Neurologic Exam

18. neurologic exam

19. neurologic exam

20. Differentials

21. Cerebral Palsy

22. Down’s Syndrome

23. Infantile Multiple Sclerosis

24. Infantile Parkinsonism

25. Muscular Dystrophies

26. Congenital Malformations

27. Metabolic Diseases

28. Metabolic Diseases

29. Spinal Cord Lesions

30. Cerebral Palsy

35. Describes a group of disorders of movement and posture, limiting activity, attributed to non-progressive underlying brain pathology. • The motor disorders of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, or by a seizure disorder. • Brain lesions of CP occur from the fetal or neonatal period to up to age 3 years Cerebral Palsy

36. The etiology of CP is not well understood and is thought to be associated with prenatal, perinatal, or postnatal events of varying causes. • Risk factors for CP are multifactorial. • Prevalence • In developed countries: about 2-2.5 cases per 1000 live births • In developing countries: about 1.5-5.6 cases per 1000 live births. Cerebral Palsy

37. Clinical Presentations of Cerebral Palsy

38. Failure to meet expected developmental milestones or failing to suppress obligatory primitive reflexes. • Abnormalities in muscle tone. • Definite hand preference before age 1 year is a red flag for possible hemiplegia. • Asymmetric crawling or failure to crawl also may suggest cerebral palsy. Clinical Presentation

39. Joint contractures secondary to spastic muscles • Hypotonic to spastic tone • Growth delay • Persistent primitive reflexes • Gait pattern abnormalities • Hip - Excessive flexion, adduction, and femoral anteversion • Knee - Flexion and extension with valgus or varus stress occur. • Foot - Equinus, or toe walking, and varus or valgus of the hindfoot Clinical Presentation

40. Spastic (70-80%) – Increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response • Dyskinetic (10-15%) – Fluctuating tone, rigid total body involvement by definition. Persistent primitive reflex patterns (asymmetric tonic neck reflex, labyrinthine) • Ataxic (<5%) – characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement Types of CP

41. Mixed - no single specific tonal quality predominating; mixture of spastic and dyskineticcomponents • Hypotonic - truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare Types of CP

42. Sensory: • Sensorineural and conductive hearing loss • Impaired visual acuity • Oculomotor dysfunction • Strabismus • Cortical visual impairment • Somatosensory impairments • Neurologic: • Seizures • Hydrocephalus Associated Conditions

43. Cognitive and linguistic: • Mental retardation • High incidence of language and learning disabilities • Dysarthria • Attention deficit hyperactivity disorder • Sleep and behavioral disturbances • Musculoskeletal: • Contractures • Hip dislocation • scoliosis Associated Conditions

44. Cardiorespiratory: • Upper airway obstruction • Aspiration pneumonitis • GI/Nutritional: • Poor growth • Gastroesophageal reflux • Constipation • Dysphagia Associated Conditions

45. Pathophysiology of Cerebral Palsy

46. <10% children with CP: evidence of intrapartum asphyxia • Associated with increased risk of CP in normal birthweight infants: intrauterine exposure to maternal infection • Prevalence of CP is increased among low birthweight infants, particularly those weighing <1,000 g at birth : because of intracerebralhaemorrhage and periventricular leukomalacia (PVL) Pathophysio

47. Believed to be caused by non-progressive disturbances in the immature and still developing fetal or infant brain • Insult to immature brain (before birth to postnatal period)  if immediately after postnatal period may be due to hypoxic-ischemic encephalopathy  cerebral insult  altered muscle tone, muscle stretch reflexes, primitive reflexes, postural ractions • Cerebral insults may be vascular, hypoxic-ischemic, metabolic, infectious, toxic, teratogenic, traumatic, and genetic in nature Pathophysio

48. Classification of Cerebral Palsy

49. Spastic Hemiplegia • focal cerebral infarction secondary to intrauterine or perinatal thromboembolism related to thrombophilic disorders, especially anticardiolipin antibodies, is an important cause • Spastic Diplegia • the most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule Pathophysio

50. Spastic Quadriplegia • most severe form of CP; swallowing difficulties are common as a result of supranuclear bulbar palsies, often leading to aspiration • the most common lesions seen are severe PVL and multicystic cortical encephalomalacia • Athetoid/Chorioathetoid/ ExtraoyramidalCP • if secondary to acute intrapartum near-total asphyxia is associated with bilateral symmetric lesions in the posterior putamen and ventrolateral thalamus Pathophysio