CEREBRAL PALSY

1
CEREBRAL PALSY

• Dr. Maninder S. Dhaliwal

2
Define

• Is defined as a
• Persistent but not unchanging
• Disorder of movement, tone and posture
• Due to non-progressive defect/lesion
• Of immature brain ( fetal life,
  infancy, childhood)
• ( immature brain cut off take as 5 yrs AAP)

3

• 5) Commonly associated with a spectrum of
  developmental disabilities such as
• Mental retardation (60)
• Epilepsy (33)
• Visual , hearing (deafness-10) and speech
  defects
• Strabismus(50)
• Cognitive dysfunction
• Sensory problems
• Emotional and behavioral problems.

4

• EXCLUDING progressive pathology and lesion of
  spinal cord.
• INCLUDING non-progressive genetic d/s or cong
  malformation

5
(No Transcript)
6
(No Transcript)
7
CLASSIFICATIONS

• TOPOGRAPHIC
• MONO
• HEMI
• DIPLEGIA
• QUADRI
• DOUBLE HEMIPLEGIA
• TRIPLEGIA

• PHYSIOLOGY
• SPASTIC
• EXTRAPYRAMIDAL
• ATAXIC
• MIXED
• ATONIC

8
(No Transcript)
9

• FUNCTIONAL
• CLASS 1 NO limitation of activity
• CLASS 2 Slight limitation
• CLASS 3 Moderate limitation
• CLASS 4 No useful physical activity

10
(No Transcript)
11

• Site of brain injury
• Cortical
• Sub cortical
• Periventricular
• Basal ganglia
• Cerebellum
• Brain stem

• Pathological
• Periventricular leucomalacia spastic diplegia
• Stroke in utero - hemiplegia
• Multifocal encephalomalacia -quadriplegia
• Cerebellar - ataxic
• Basal ganglia, thalmus, putamen - dyskinetic

12
(No Transcript)
13
(No Transcript)
14
(No Transcript)
15
(No Transcript)
16
(No Transcript)
17
(No Transcript)
18
(No Transcript)
19
(No Transcript)
20
(No Transcript)
21
(No Transcript)
22
(No Transcript)
23
(No Transcript)
24
(No Transcript)
25
Severity

• Motor development quotient
• motor age / chronological age 100
• gt70 minimal deviancy without delay
• 50-70 mild motor disorder
• lt50 classical CP

26
Etiological

• Prenatal
• I, iron def.,poor nut.
• Inf, UTI, high fever
• Chorioamniotis
• HTN, DM
• Teratogens
• Poor ANC
• LOW SES
• Twins
• Fetal vasculopathy

• Perinatal
• Birth asphyxia
• Premature / LBW
• IUGR
• Hyperbilirubenemia
• IVH
• Sepsis, pneumonia, meningitis
• Develop. malformation

• Postnatal
• CNS infections
• Head injuries
• Seizures
• Hypoxic damage
• Hyperpyrexia damage

27
(No Transcript)
28
(No Transcript)
29
(No Transcript)
30
Early markers of CP

• SLOW head growth
• Poor head control
• Eye roving eyes, poor hand regard, persistent
  squint.
• Ear lack of auditory response
• Irritability, seizures, poor suck, poor quality
  of sleep.
• Extreme sensitivity to light

• Cortical thumb beyond 8 weeks
• Handedness before 2 yrs
• Paucity of limb movements
• Scissoring of lower limbs
• Toe walking
• Abnormal tone
• Persistence of primitive reflexes or failure to
  acquire postural reflexes
• Stereotypic abnormal movements
• Lack of alertness

31
(No Transcript)
32
(No Transcript)
33
(No Transcript)
34
(No Transcript)
35
(No Transcript)
36
(No Transcript)
37
(No Transcript)
38
(No Transcript)
39
(No Transcript)
40
(No Transcript)
41
(No Transcript)
42
(No Transcript)
43
(No Transcript)
44
(No Transcript)
45
(No Transcript)
46
 Differential diagnosis

• In the early infancy when the child is in
  hypotonic phase, neuromuscular conditions like
  myopathies may cause diagnostic confusion.
• Children with mental retardation may have
  hypotonia but do not have abnormal motor patterns
  or postures

47

• Neurodegenerative conditions which have onset in
  early infancy such as Tay-Sach disease, Krabbe's
  disease, and Metachromatic Leucodystrophy can
  mimic CP.
• The progressive course of these conditions can be
  ascertained on the basis of history, and relevant
  investigation can confirm the diagnosis.

48

• Dopa-responsive dystonia and organic aciduria
  like glutaric aciduria may look like dystonic CP.

49

• Assessment of General Health
• Growth, and nutritional disorders
• Frequent respiratory tract infections are common
  because of ineffective cough reflex.
• Facial dysmorphism and other congenital anomalies
  should be noted.
• Skin should be inspected for neurocutaneous
  stigmata.
• Head circumference should be noted and plotted .

50

• Reliable measures of length may be difficult to
  obtain due to concomitant contractures or
  scoliosis.
• Alternate measures to length such as segmental
  measures of upper arm and lower leg are sometimes
  used.
• Skinfold thickness is a useful and a less
  cumbersome method of assessing nutritional
  status.

51

• Neurological Evaluation
• The history of previous developmental milestones
  should be obtained for all domains of development
  i.e. gross fine motor, cognitive, speech and
  language and socialization.

52

• A thorough neurological evaluation should be
  performed which includes assessment of cranial
  nerves, posture, muscle tone of extremities,
  trunk and neck, deep tendon reflexes postural
  response and primitive reflexes .
• Physical examination should include the
  observation of child in prone, supine, sitting,
  standing, and if appropriate in walking and
  climbing positions.

53

• This is followed by assessment of the current
  level of functioning in all these domains and
  assessment of self help and adaptive skills in
  daily activities such as feeding, dressing,
  brushing teeth .

54
Disorder in Movement and Posture

• Among the most clinically useful primitive
  reflexes are Moro, Tonic labyrinthine and ATNR
• Postural reactions are sought in each of 3
  categories righting, protection and equilibrium.

55
Muscle Tone

• Abnormality of tone is an integral part of CP.
• Hypertonia in CP may be purely due to spasticity
  (pyramidal ) or else due to dystonia
  (extrapyramidal).

56
Assessment of Cognition and Behavior

• Metal retardation was found the commonest
  associated problem in children with CP
• Conventional tests of intelligence may prove
  erroneous in children with CP because of motor
  and communication deficits.
• Age appropriate non-verbal intelligence tests
  have to be administered for this purpose.

57
Assessment of Vision and Hearing

• In children with hearing impairment with
  associated microcephaly and congenital heart
  disease should be looked for other stigmata of
  TORCH infection.
• In cases with dyskinetic CP, presence of hearing
  impairment may point to kernicterus as a cause of
  CP.
• Sensorineural hearing loss is a prominent feature
  of CP due to Iodine deficiency in endemic areas.

58
Assessment of Speech and Language

• These may be due to hearing impairment, cognitive
  deficits, or oromotor dysfunction
• Difficulty in communication by language or
  gestures further compound behavior problems.

59
Assessment of Feeding and Nutrition

• Oromotor dysfunction, inability to self feed ,
  and inability to request for food due to
  communication disorder result in feeding problems
  and poor nutritional status in children with CP .
• Gastro-esophageal reflux or choking/coughing
  while feeding which may further cause aversion to
  food .

60

• Child should also be assessed for tongue thrust
  or tonic bite, hypersensitivity to touch,
  drooling of saliva and dental hygiene.

61
Orthopedic Problems

• Hip subluxation, scoliosis, equinus deformity,
  and contractures of hamstring muscles and
  tendoachilles.
• Reduced bone density and propensity to fractures
  with trivial trauma is common
• Equinus deformity is the most common
  musculoskeletal abnormality in patients with CP.
• It is due to fixed or spastic contracture of
  gastrocnemius and causes the typical tip toe or
  toe heel gait in children with CP.

62

• Epilepsy Epilepsy is more common in children
  with CP. In a population based study 38 of
  children with CP had epilepsy
• Children with CP caused by CNS malformations, CNS
  infection, and grey matter damage have been
  reported to show a higher frequency of epilepsy
  than children with CP of other aetiology, and
  also had less chance of becoming seizure-free

63

• Role of Neuroimaging
• Neuroimaging (MRI preferred to CT) is recommended
  in children with cerebral palsy in order to
  establish structural brain abnormality which may
  further help in finding the etiology and giving
  prognosis

64

• Disabling conditions be evaluated on multiple
  axes-
• Pathophysiology (underlying disease)
• Impairment (clinically observable abnormality)
• Functional limitations (effect on task
  performance)
• Disability (effect on daily living) and
• Societal limitations (effect on life time
  opportunities).
• Since CP is a changing disorder it is evident
  that some limitations may not be evident early in
  life but manifest in the school age or later.

65

• Assessment of Home Situations Evaluation is not
  complete without the assessment of the home
  situation such as family size, financial
  resources and family support.

66

• Child rearing conditions that support and enrich
  early development can compensate for many
  biological deficits.
• On the other hand, poverty, illiteracy in
  parents, large family size, frequent change in
  residence, non-availability of special
  rehabilitation centers may deprive the child of
  appropriate care.

67

• The hemiplegic type of CP has the best prognosis
  for walking with 95 walking by 3 years compared
  to 40 of other groups.
• Age of sitting is a good guide to prognosticate
  about walking. A child who is able to sit
  unsupported at 2 years will eventually be able to
  walk.
• On the contrary, a child whose sitting is
  delayed beyond 3 years has remote prospects for
  functional outdoor walking

68

• Management - The pediatrician's initial role
  consists of making a correct diagnosis,determining
  the etiology, and identification of the type,
  extent and severity of the neuromotor deficit as
  well as of associated problems

69
(i) Breaking the news
70
ii) Comprehensive assessment

• a multidisciplinary team comprising of a
  neuro-developmental pediatrician as the
  team-leader,
• physiotherapist,
• occupational therapist,
• clinical psychologist,
• speech pathologist,
• orthopedic surgeon,
• otorhinolaryngologist,
• ophthalmologist,
• teacher,
• play therapist and
• social worker is required,
• preferably under one roof.

71
(No Transcript)
72
Physiotherapy (P.T.) -

• P.T. especially when started early in life, is
  helpful in promoting normal motor development,
  and preventing deformity and contractures.
• In the young child it aims at reducing abnormal
  patterns of movement and posture and promoting
  the normal ones so as to enable the child to gain
  maximal functional independence.
• A number of techniques have been used for this
  purpose. The neurodevelopmental Bobath technique
  is commonly used.

73

• It consists of guiding the child through normal
  sequences of motor development,
• inhibition of primitive and abnormal reflexes,
• re-inforcement of normal postural reflexes and
• facilitation of normal movements.

74
(No Transcript)
75
Occupational Therapy

• The role of P.T. and O.T. are so closely linked
  that they could infact be considered together.
• The occupational therapist is usually better
  trained to advise on activites of daily living
  like feeding, bathing, dressing, toilet training
  etc, and the equipment needed to facilitate
  these.
• Co-ordination and sensory-perceptual integration
  can be taught and multisensorial stimulation
  provided through peg board, blocks and other toys
  of different colours, textures, sizes and shapes,
  and producing different sounds

76
(No Transcript)
77

• Play Therapy It is the use of a natural activity
  with a young child, to help him consolidate the
  levels of development that he has reached and
  encourage him to move on, to the next level.
• Parents are taught to break down each activity
  into its simplest components and make the child
  practice it in a real life situation. It then is
  not considered as an 'exercise' but becomes a way
  of life.

78
(No Transcript)
79
(No Transcript)
80
(No Transcript)
81
(No Transcript)
82
(No Transcript)
83
Assistive and adaptive devices

• Various simple modifications like angled spoons,
  two handled cups etc can be made to help the
  child.
• Old stools and boxes can be adapted to provide
  support during sitting
• Parallel bars can be constructed with logs of
  wood to help gait training.
• Standing frames and prone boards are a useful
  intermediate stage in mobilization.

84
(No Transcript)
85

• A number of high technology devices like
  programmed wheel chairs, electronic feeding
  devices, various access systems, computerized
  speech systems and cochlear implants are
  available

86

• Splints, Casts and Calipers Specially designed
  shoes, ankle-foot orthoses (AFO) and calipers
• AFOs, are particularly useful in children with
  spastic diplegia who have dynamic spasticity with
  tendo- achilles (TA) tightening.

87
Management of Spasticity

• Proper P.T. given regularly considerably reduces
  spasticity and improves function.
• (i) Drugs
• Baclofen - acts at the level of spinal cord
  neurons and enhances GABA activity.
• It is commonly used in a starting dose of 1.25 -
  2.5 mg BD orally and increased gradually upto a
  maximum of 30 mgm/day, monitored by a clinical
  response.
• It is not recommended in children with seizures
  as it may provoke them. (

88

• ii) Diazepam- a small dose given half an hour
  before PT is effective in some cases especially
  where anxiety increases spasticity. Its
  disadvantage is that it may cause unacceptable
  drowsiness.
• (iii) Tizanidine an alpha 2 adrenergic agent and
• (iv) Dantrolene sodium which acts on calcium
  channels have also been used, but experience with
  them is limited.

89

• Baclofen has also been used intrathecally using
  implantable infusion pumps.
• It may be helpful in cases of severe spasticity
  or disabling total body dystonia

90

• Botulinum Toxin (BTA) is derived from
  Claustridium botulinum.
• It causes muscle relaxation by blocking the
  release of acetylcholinesterase, with loss of
  motor end plates.
• As affected nerve roots sprout to form new
  junctions, the relaxing effect reverses over 3-6
  months.
• It is more often used in children with spastic
  diplegia.

91

• Surgery Surgery is useful in some children with
  spasticity, especially where mainly the lower
  limbs are involved.
• Tendon lengthening and transfer and arthrodesis
  are some of the procedures commonly performed.
• Generally multilevel surgery is required and is
  done after 8 years of age.
• Simultaneous availability of intensive
  physiotherapy is essential.

92

• Dorsal rhizotomy which involves selective
  resection of posterior nerve roots from L 2_ to S
  2
• It may be helpful in children with severe lower
  limb spasticity, with sufficient trunk control
  and some form of forward locomotion.
• Its advantage must be weighed carefully against
  the sensory losses that may occur after the
  procedure.

93

• Relief of athetosis and dystonia - is difficult
  occasionally levo-dopa for severe athetosis and
  carbamezepine for dystonia may be helpful.
• Thalamotomy for athetoid CP, stereotactic
  dentatomy and chronic cerebellar stimulation via
  implanted electrode .

94
Management of Associated Problems.
95

• Parent Counselling This is one of the most
  important aspects because parents are pivotal in
  the management of their child.
• It is an ongoing process, as the parents need to
  be counseled periodically at various stages of
  their child's development.