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CEREBRAL PALSY

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Physiotherapy (P.T.) - P.T. especially when started early in life, is helpful in promoting normal motor development, and preventing deformity and contractures. – PowerPoint PPT presentation

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Title: CEREBRAL PALSY


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CEREBRAL PALSY
  • Dr. Maninder S. Dhaliwal

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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)

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  • 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.

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

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CLASSIFICATIONS
  • TOPOGRAPHIC
  • MONO
  • HEMI
  • DIPLEGIA
  • QUADRI
  • DOUBLE HEMIPLEGIA
  • TRIPLEGIA
  • PHYSIOLOGY
  • SPASTIC
  • EXTRAPYRAMIDAL
  • ATAXIC
  • MIXED
  • ATONIC

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

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  • 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

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

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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

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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

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 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

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  • 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.

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

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  • 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 .

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  • 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.

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  • 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.

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  • 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.

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  • 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 .

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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.

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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).

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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.

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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.

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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.

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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 .

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

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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.

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  • 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

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  • 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

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  • 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.

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

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  • 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.

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  • 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

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  • 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

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(i) Breaking the news
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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.

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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.

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  • 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.

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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

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  • 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.

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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.

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

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  • 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.

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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. (

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  • 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.

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

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  • 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.

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  • 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.

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  • 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.

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  • 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 .

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Management of Associated Problems.
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  • 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.
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