Cerebral palsy


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Cerebral palsy

  2. 2. DEFINITION Cerebral Palsy is a STATIC encephalopathy and may be defined as a non-progressive disorder of posture and movement, often associated with epilepsy and abnormality of speech, vision and intellect resulting from a defrct or lesion of the developing brain.
  3. 3. MAGNITUDE OF THE PROBLEM • Cerebral palsy is the most common cause of chronic motor disability that begins in childhood. • It is difficult to estimate the precise magnitude of the problem since milder cases are likely to be missed.
  4. 4. • It is the most common childhood physical disability and affects 2 to 2.5 children per 1,000 born in the United States. • The exact incidence and prevalence from our country is not known.
  5. 5. ETIOLOGY Idiopathic (no known cause of damage to brain during pregnancy) - is the most common cause. CP could be classified on the basis of underlying cause of cerebral palsy- Prenatal causes Perinatal causes Postnatal causes
  10. 10. POSTNATAL CNS INFECTIONS (MENINGITIS, ENCEPHALITIS) HYPOXIA SEIZURES COAGULOPATHIES NEONATAL HYPERBILIRUBINEMIA Head trauma from shaken baby syndrome (child abuse) during the 1ST year of life Lead poisoning during the 1st two years of life
  11. 11. PHYSIOLOGICAL CLASSIFICATION Physiological classification This is determined on the basis of the nature of the movement disorder, muscle tone, and anatomic distribution. A single patient may have more than one type. Spastic CP is the most common, accounting for about two thirds of cases.
  12. 12. Pyramidal (or spastic) CP: This type is characterized by neurologic signs of upper motor neuron damage with increased "clasp knife" muscle tone, increased deep tendon reflexes, pathologic reflexes, and spastic weakness.
  13. 13. Extrapyramidal (nonspastic or dyskinetic) CP: This type is characterized by prominent involuntary movements or fluctuating muscle tone, with choreoathetosis as the most common subtype. Distribution is usually symmetric among the four limbs.
  14. 14. • Hypotonic CP: This type manifests as generalized muscle hypotonia that persists with normal or increased deep tendon reflexes. Many patients with this condition develop cerebellar deficits of incoordination and ataxia, and about one third of patients have severe retardation. • Ataxic CP: Primarily cerebellar signs are seen. • Mixed types
  15. 15. TOPOGRAPHICAL CLASSIFICATION This is based on which limbs are involved. • Monoplegia – Only one limb is involved. • Paraplegia – Both the legs are involved • Hemiplegia – Both upper and lower limbs are involved on one side • Triplegia – Three limbs are involved • Quadriplegia – all the four limbs are involved • Diplegia – The lower limbs are more involved than the upper limbs.
  17. 17. EARLY SIGNS OF CEREBRAL PALSY Paucity of movement or excessive & disorganized movements Stereotyped behaviour Persistent tone abnormalities Feeding problems Hyperextension of head & neck Consistent fisting of hands beyond 2 months Delayed social smile Reduced growth of head size Early sign of hand preference in the 1st yr of life Persistent moro’s reflex beyond 6months of age
  18. 18. ETIOLOGY SPASTIC DIPLEGIA • Prematurity • ischemia • infection • hemorrhages • LBW Neuropathology: • periventricular leucomalacia SPASTIC QUADRIPLEGIA • Ischaemia • Infections • genetic/developmental • birth asphyxia / trauma Neuropathology : • PVL • multicystic malformation
  19. 19. HEMIPLEGIA • Thrombophilic disorders, • infections, PVH • Neuropathology: stroke in utero or neonatal EXTRA PYRAMIDAL • Kernicterus, • mitochondrial, genetic/ metabolic • neuropathology: basal ganglia, putamen ,globus pallidus , thalamus
  21. 21. SPASTIC HEMIPLEGIA • Involvement of the arm and leg on one side of the body • Upper extremity more severly involved • Constitutes 20% of cases with spastic CP • Manifests from 4th month – 2nd yr of life • Incomplete Moro’s or Premature development of handedness could be early symptom
  22. 22. • M/C form of CP • Functional prognosis good as compared to other forms • Underdevelopment of affected side is common • Will crawl with affected arm tucked under the trunk and pushing self with the sound arm • Cortical thumb
  23. 23. DIPLEGIA
  24. 24. DIPLEGIA • Patients with diplegia have motor abnormalities in all four extremities, with the lower extremities more affected than the upper. • The close proximity of the lower extremity tracts to the ventricles most likely explains the more frequent involvement of the lower extremities with periventricular lesions.
  25. 25. • The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four-limbed crawling movement. • If the spasticity is severe, application of a diaper is difficult because of the excessive adduction of the hips.
  27. 27. SPASTIC QUADRIPLEGIA • Spastic quadriplegia is the most severe form of CP because of marked motor impairment of all extremities and the high association with mental retardation and seizures. • In quadriplegia, all four extremities are equally involved, and many patients have significant cognitive deficiencies that make care more difficult. • Head and neck control are usually present, which helps with communication, education, and seating.
  28. 28. ATHETOID CP • Athetoid cerebral palsy is less common than spastic cerebral palsy. • This type is most likely to be associated with birth asphyxia.
  29. 29. • It is caused by an injury to the extrapyramidal tracts and is characterized by dyskinetic, purposeless movements that may be exacerbated by environmental stimulation. • The clinical picture varies based on the level of excitement of the patient. In pure athetoid cerebral palsy, joint contractures are uncommon.
  30. 30. • Affected infants are characteristically hypotonic with poor head control and marked head lag and develop increased variable tone with rigidity and dystonia over several years. • Feeding may be difficult, and tongue thrust and drooling may be prominent. • Speech is typically affected because the oropharyngeal muscles are involved. • Seizures are uncommon, and intellect is preserved in many patients.
  31. 31. DYSTONIC CP • The trunk muscles and the proximal portion of the limb muscles are predominantly affected. • Movements may be slow and persistent. • Abnormal movements invoving the head and neck area.
  32. 32. ATONIC CP • Hypotonicity of lower limb muscles. • Arms may have near normal strength and coordination. • Often associated with slow attainment of motor milestones.
  33. 33. MIXED CP • Manifestations of both spastic and extrapyramidal types. • Often an ataxic component is present. • Have mild to moderate degree of choreoathetosis.
  35. 35. Cerebral palsy can be diagnosed using Levine (POSTER) criteria. POSTER stands for – P - Posturing/abnormal movements O - Oropharyngeal problems (e.g., tongue thrusts, swallowing abnormalities) S - Strabismus T - Tone (hyper- or hypotonia) E - Evolutional maldevelopment (primitive reflexes persist or protective equilibrium reflexes fail to develop R - Reflexes (increased deep tendon reflexes/persistent Babinski's reflex)
  36. 36. • In a child with cerebral palsy motor deficit is always present. • The usual presenting complaint is that child is not reaching motor milestones at the appropriate age. • Medical history establishes that the child is not losing function. • This history, combined with a neurologic examination establishing that motor deficit is due to a cerebral abnormality, establishes the diagnosis of cerebral palsy.
  37. 37. • Serial examinations may be necessary if the history is unreliable. • Other causes of neonatal hypotonia include muscular dystrophies, spinal muscular atrophy, Down's syndrome, spinal cord injuries.
  38. 38. Laboratory tests are not necessary to establish the diagnosis. Workup is helpful for assessment of recurrence risk, implementation of prevention programs, and medico legal purposes.
  39. 39. Metabolic and genetic testing should be considered if on follow-up the child has - • Evidence of deterioration or episodes of metabolic decompensation • No etiology determined by neuroimaging • Family history of childhood neurologic disorder associated with cerebral palsy • Developmental malformation on neuroimaging
  40. 40. • An EEG should be obtained when a child with cerebral palsy has a history suggestive of seizures. • Children with cerebral palsy should be screened for ophthalmologic and hearing impairments, speech and language disorders. • Nutrition, growth, and swallowing function should be monitored.
  41. 41. • Neuroimaging is recommended if the etiology has not been established previously; for example, by perinatal imaging. • MRI, when available, is preferred to CT scanning because of higher yield in suggesting an etiology, and timing of the insult leading to cerebral palsy.
  42. 42. What other conditions are associated with cerebral palsy?
  43. 43. Neurological problems associated with cerebral palsy include: • Seizures and epilepsy ( As many as half of all children with CP have one or more seizures.) • Behavior problems • Mental retardation ( Mental impairment is more common among those with spastic quadriplegia than in those with other types of cerebral palsy.)
  44. 44. • Learning disabilities • Hearing loss • Attention deficit–hyperactivity disorder • Speech impairment • Swallowing difficulties • Hydrocephalus
  45. 45. Visual impairment As many as 75 to 90 percent of children with CP may suffer from- • strabismus, • Amblyopia (“lazy eye”), • optic atrophy • nystagmus • visual field defects • refractive errors
  46. 46. The secondary effects of cerebral palsy include: • Poor growth • Sleep disorders • Poor nutrition • Upper airway obstruction • Aspiration pneumonia
  47. 47. • Communication disorder • Gastroesophageal reflux • Tooth decay and gum disease • Hernia • Constipation • Bladder control problems • Drooling • Spinal deformities and osteoarthritis (Spinal deformities can make sitting, standing, and walking difficult and cause chronic back pain. Pressure on and misalignment of the joints may result in osteoporosis)
  48. 48. • Contractures. Muscles can become painfully fixed into abnormal positions, called contractures, which can increase muscle spasticity and joint deformities in people with CP. • Frequent fractures
  49. 49. MANAGEMENT
  51. 51. • These care providers are roughly organized around the educational system, primary medical care provider, the cerebral palsy specialized medical team, and community support services. Significant overlap and good communication provide the best resources to the child and the family.
  52. 52. • Because of the heterogeneous nature of cerebral palsy, it is difficult to make generalized statements regarding treatment, and it is best to have an individualized approach to each patient and his or her needs.
  53. 53. Four basic treatment principles exist - • The first is that although the central nervous system injury, by definition, is nonprogressive, the deformities caused by abnormal muscle forces and contractures are progressive. • The second, which can be a source of frustration, is that the treatments currently available correct the secondary deformities only and not the primary problem, which is the brain injury.
  54. 54. • The third is that the deformities typically become worse during times of rapid growth. For some patients, it may be beneficial to delay surgery until after a significant growth spurt to decrease the risk of recurrence. • The fourth is that operative or nonoperative treatment should be done to minimize the impact it has on the patient's socialization and education.
  55. 55. HOME MODIFICATIONS There are various ways to modify the home environment so that your child will have more independence and things will be easier on parents too. Example- • Replace doorknobs with lever door handles. • Replace entrance steps with ramps. • Install a hinged arm support for help with toileting. • Rearrange furniture in order to remove obstructions.
  56. 56. CHOOSING APPROPRIATE SEATING Strollers, feeding chairs, tumble form sets, headrests, seating supports, and corner chairs—all of these are forms of adaptive seating that may benefit children with cerebral palsy.
  57. 57. PRESSURE MANAGEMENT AWARENESS For children who use wheelchairs, the need to change positions or relieve pressure, particularly on their buttocks or ischial tuberosities, is critical. If is not relieved, skin breakdown or pressure sores can occur.
  58. 58. If the child is not standing between 18 and 24 months, it is necessary to start a child standing even when she does not have adequate head or upper body control to stand alone.
  59. 59. Standing is important because it allows the child to do some weight bearing through the legs, which in turn helps make the bones stronger and stimulates the development of motor coordination and head control.
  60. 60. WALKERS AND GAIT TRAINERS • There are no absolute indicators for initiating the use of a walker. • If child is able to hold up head independently, sitting in a chair with minimal support, and being able to stand and accept weight through the legs are a few of the basics that are needed.
  61. 61. REHABILITATION TECHNIQUES Neurodevelopmental Therapy • These specialized techniques encourage active use of appropriate muscles and diminish involvement of muscles not necessary for the completion of a task.
  62. 62. • Improvements in efficiency can include decreased energy used during a task, decreased work required of the muscles during a task, and habituation of new patterns of movement. • Occupational, speech, and physical therapists as well as educators can use NDT.
  63. 63. STRENGTHENING EXERCISES Muscle strength is related to motor performance and should be an integral part of a rehabilitation program that addresses other impairments which inhibit motor performance in this population, such as muscle–tendon shortening, spasticity, and coordination deficits.
  64. 64. ELECTRICAL STIMULATION TECHNIQUES • Neuromuscular electrical stimulation (NMES) • Transcutaneous electrical stimulation (TES)
  65. 65. HIPPOTHERAPY The North American Riding for the Handicapped Association (NARHA) has defined hippotherapy as “The use of the movement of a horse as a tool by therapists to address impairments, functional limitations and disabilities in patients with neuro musculoskeletal dysfunction.
  66. 66. AQUATIC THERAPY • The relief of hypertonus in the spastic type of CP is one of the major advantages of aquatic therapy. • When a body is immersed in warm water (92°to 96°F), its core temperature increases, causing reduction in gamma fiber activity, which in turn reduces muscle spindle activity, facilitating muscle relaxation and reducing spasticity, thus resulting in increased joint range of motion and consequently creating better postural alignment.
  67. 67. A great way to start gait training, especially after surgical procedures, is pool walking. This means the pool needs to have handles available in the water at the correct height.
  68. 68. M.O.V.E. (MOBILITY OPPORTUNITIES VIA EDUCATION) CURRICULUM • It is an activity-based curriculum designed to teach individuals basic functional motor skills needed for adult life. • The MOVE curriculum provides a framework for teaching the skills necessary for individuals with disabilities to gain greater physical independence.
  69. 69. • Physical and occupational therapies are useful for promoting mobility and the use of the upper extremities for activities of daily living. • Speech language pathologists promote acquisition of a functional means of communications.
  70. 70. • Communication skills may be enhanced by the use of Bliss symbols, talking typewriters, electronic speech generating devices, and specially adapted computers including artificial intelligence computers to augment motor and language function.
  71. 71. MEDICAL MANAGEMENT • Several oral medications have been used to reduce tone, including diazepam, baclofen, dantrolene, tizanidine, and clonidine. • Although they can decrease spasticity, their sedating side effects are not well tolerated in Children. • Intrathecal baclofen has been shown to reduce spasticity with fewer side effects.
  72. 72. INTRATHECAL BACLOFEN PUMPS • Use of intrathecal baclofen in the pediatric patient having CP has yielded as good a reduction in tone as dorsal rhizotomy and does not represent an ablative procedure. This is important because, unlike rhizotomies, it is entirely reversible.
  73. 73. BACLOFEN • GABA agonist -Acts on spinal cord neurons • inhibits release of excitatory neurotransmitter at level of spinal cord • Dose : 2 mg/kg/day, can be given intrathecally • ADR : hypotonia of trunk muscles, dec alertness, dec appetite, vomiting, aggravates epilepsy • Withdrawal: Rebound spasticity
  74. 74. BOTULINUM TOXIN A (BTA) • Blocks acetylcholine release, with a loss of motor end plates • Effect – 3 to 4 months • Multiple site intramuscular injections for selected muscle groups • Effective only for dynamic spasticity • Helps delay surgery beyond 8 yrs of age • ADR : painful injection • systemic effects- laryngeal weakness, bladder incontinence
  75. 75. SURGICAL TECHNIQUES Upper Extremity Procedures 1. Shoulder Adductor, Extension, and External Rotator Lengthening 2. Humeral Derotation Osteotomy (in patients with severe abduction external rotation contractures) 3. Elbow Flexion Contracture Release 4. Pronator Release or Transfer 5. Flexor Carpi Ulnaris Transfer for Wrist Flexion Deformity 6. Proximal Row Carpectomy and/or Wrist Fusion 7. Thumb Adductor Lengthening
  76. 76. A rhizotomy procedure in which the roots of the spinal nerves are divided produces considerable improvement in selected patients with severe spastic diplegia.
  77. 77. SPINAL PROCEDURES 1. Posterior Spinal Fusion with Unit Rod 2. Anterior Spinal Release
  78. 78. • Hip Procedures- Iliopsoas Lengthening, Adductor and Iliopsoas Lengthening with Proximal Hamstring Lengthening. • Knee Operative Procedures- Hamstring Lengthening • Foot and Ankle Procedures- Gastrocnemius Lengthening, Tendon Achilles Lengthening
  79. 79. MANAGEMENT OF ASSOCIATED CONDITIONS Strabismus is normally treated by correcting the visual acuity in each eye, either with glasses or by patching. If a significant strabismus remains even after these therapies, then surgery is indicated.
  80. 80. Feeding – Problems: Tongue thrusting Drooling of saliva Risk of aspiration GERD Mgt: Upright position during and after feeds Thickening of feeds Drugs - antacids, prokinetics, H2 blockers NGT feeds Antireflux surgery – fundoplication For sialorrhea - glycopyrrolate
  81. 81. • Constipation – Risk of acquired megacolon Increased fluid intake, varied diet, lactulose, enema • Incontinence – Patient toilet training may be attempted according to stage of development