Case: Children with Disability

1. Case: Children with Disability

2. Case • J.R. 3 y/o boy • Stiffness when crying • Tiptoe walking

3. Maternal and Birth History • NSD, full term • APGAR score: 5 BW: 2.5kg • 35y/o G1P1 (1-0-0-1) • Regular pre-natal check-up with O.B. • Mother (+) hx of UTI during last trimester, no bleeding • Upon delivery, cord was coiled on the neck 3x

4. Developmental History

5. Pertinent PE Findings • Ambulatory with scissoring gait • Poor balance • Maintains high guard of upper extremity • (+) cortical thumb • With good head control • (+) drooling • Good sitting balance • (+) tightness of both Achilles tendons • (+) clonus

6. Diagnosis: Cerebral Palsy secondary to perinatal brain injury

7. What are the pertinent facts in the history that may point or contribute to your working diagnosis?

8. Salient Features • 3y/o boy – stiffness when crying and tiptoe walking • Delay in achieving developmental milestones • Birth weight: 2.5 kg (LBW) • Cord coiled on the neck 3x • Ambulatory with scissoring gait • Poor balance • Maintains high guard of extremities • (+) cortical thumb • (+) drooling • (+) tightness of both achilles tendons • (+) clonus

9. Developmental Milestones • At present (3y/o) • walks but needs assistance • Can make simple sounds • Continues to drool

10. Cerebral Palsy • collection of diverse syndromes characterized by disorders of movement and posture • non-progressive • static movement disorder (weakness possible) • delayed developmental milestones • persistence of primitive reflexes • increased motor tone/floppy

11. Cerebral Palsy Etiology • brain injury that occurs prenatal, perinatalor postnatal • brain dysfunction Incidence • 2 per 1000 live births (industrialized countries) • M>F 1.33 : 1.00

12. Cerebral Palsy Factors that Increases Incidence of CP • low birth weight <25OO g • gestational age <32 wk – most common antecedent of CP • maternal factors • mental retardation • seizure disorder • hypothyroidism • 2 or more prior fetal deaths • sibling with motor deficit • 3rd trimester bleeding

13. Cerebral Palsy Manifestations • floppy infant • persistent obligatory reflexes

14. Cerebral Palsy Diagnosis • High Risk History • Posture - FSP, ESP(flexor, extensor synergy patterns), opisthotonus, scissoring gait = d/t spasticity of hip adductors; straphanger UE • Oral Motor - tongue thrust, tonic bite • Strabismus • Tone (while awake and not struggling) • Evolution of Postural Reactions • Reflexes - MSRs, clonus, Babinski • confirmed by: MRI • Other tests: metabolic, biochemical, developmental

15. Cerebral Palsy Classification by limb affectation: • Quadriplegia – all 4 extremities + trunk • Diplegia – LE > UE, most common • Monoplegia– only 1 limb, either an arm or leg • Hemiplegia – involvement on one side of body, including arm and leg

16. Cerebral Palsy • by muscle tone: • Spastic (70-80%) • most common • signs of UMN involvement • Hypertonic • NM condition stemming from damage to corticospinal tract, motor cortex or pyramidal tract/ velocity dependent inc in muscle tone • Modified Ashworth scale->

17. Cerebral Palsy Atonic (10%) • Hypotonia and tremors • Motor skills like writing, typing, using scissors, balance while walking are affected • Visual and/or auditory processing of objects Athetoid (25%) • Alternating hyper and hypotonia • Trouble holding themselves upright, steady position of sitting or walking • Involuntary motions • Difficulty getting their hand to a certain spot (e.g. scratching nose, reaching for a cup) • May not be able to hold on to objects

18. Cerebral Palsy Comorbidities • Mental retardation (IQ<50) – 31% • Active seizures- 21% • Mental retardation (IQ <50)+ and not walking- 20% • Blindness- 11%

19. Topographical Classification of Cerebral palsy in our patient • Spastic • (+) cortical thub, (+) drooling, (+) clonus • Diplegia • ambulatory with scissoring gait, poor balance and maintains high guard of UE, good head control

20. 5. How will you grade the spasticity of the patient? (Modified Ashworth)

21. Clinical Scale For Spastic HypertoniaMODIFIED ASHWORTH SCALE

22. 6. How will you manage the spasticity?

23. Management • Rehabilitation • Daily stretching • Oral Medications • Local Injectables • Surgical treatments • Tendon release, Selective dorsal rhizotomy

24. Management • Rehabilitation • individualized stretching and exercise program that can be used at home on a regular basis • Improve ROM and function • Useful component to all other modalities • Braces, walking aids (help reduce impact of spasticity)

25. Management • Oral Medications • Baclofen (Lioresal) • Tizanidine (Zanaflex) • Diazepam (Valium) • Clonazepam (Klonopin) • Dantrolene sodium (Dantrium)

26. Management • Advantages of Oral Medications: • Relax a large number of muscles • Dose is easily adjusted • May be stopped anytime • Disadvantages: • Modest effect • Drowsiness, dizziness, weakness • Liver inflammation

27. Management • Local Injections • Phenol black • IntrathecalBaclofen pump • Botox injection

28. Management • Botilinum toxin • Protein made by bacteria causing botulinism • BTX-A (Botox), BTX-B (Myobloc) • Can relax spastic muscle when injected in small quantities • Affects only injected muscle • 2-3wks to take effect last 3-6mo. • Safe and can be injected w/o use of sedatives, however injections maybe uncomfortable

29. Management • IntrathecalBaclofen Therapy (ITB) • Baclofen pump • Surgically implanted • More effective than oral Baclofen • Delivers medication directly to the spinal fluid via catheter • Well tolerated, reversible • Test injection (to determine if patient is good candidate for the Baclofen pump)

30. 7. What will be your immediate rehab goal for this patient?

31. Goals • Obtain optimal function despite residual disability • Prevent secondary impairement or complications • Prevent or delay contractures • Maintain mobility • Improve function • Promote motor and developmental skills • Activities necessary to help the child reach his/her full potential

32. Physical Therapy: Daily ROM Exercises • Stretching exercises • Increase motion • Progressive resistance exercises • Increase muscle strength

33. Daily ROM Exercises • Strengthening knee extensor muscles helps to improve crouching and stride • Postural and motor control training • Should follow the dev’t sequence of noraml children (head and neck  trunk control) • Use of age appropriate play and of adaptive toys and games based on the desired exercises

34. Daily ROM Exercises • Hippotherapy • Horse back riding therapy • Improve child’s tone, ROM, strength, coordination, balance • Offers many potential cognitive, physical and emotional benefits

35. Occupational Therapy • Activities of Daily Living • Feeding, dressing, toileting, grooming • Goal: to function as independently as possible w/ w/o use of adaptive equipment

36. Speech Therapy • Cerebral Palsy • Involvement of the face and oropharynx, causing dysphagia, drooling and dysarthria • Speech therapy can be implemented to help control the muscles of the mouth and jaw and improve communication

37. Encourage se of age appropriate play and of adaptive toys and games based on the desired exercises to achieve child’s cooperation

38. 8. Will he need assistive device?

39. Assistive Devices • Ankle foot orthoses (AFO) • Help improve balance and walking • Hinge AFO more effective and helpful • Reduction in foot pronation and prevent contracture by stretching spastic muscles • Splints • Correct spasticity in the hand muscles

40. Assistive Devices • Devices that help individuals move about more easily and communicate successfully at home at school, or in workplace • Help child with CP to overcome physical and communication limitations

41. Assistive Devices • Postural support or seating systems • Open front walkers • Quadrapedal canes (lighweight or metal) • Gait poles