CEREBRAL PALSY

PRESENTED BYDr. Libin Thomas Manathara

HISTORY• The term cerebral palsy was first used in 1843 by the

English orthopedic surgeon William Little in a series of lectures entitled “Deformities of the Human Frame”

• It was known for many years as “Little’s disease”

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Dr. William John Little

William John Little (1810 –1894) was an English surgeon who is credited with the first medical identification of spastic diplegia, when he observed it in the 1860s amongst children

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Sir William Osler, (July 12, 1849 – December 29, 1919) was a Canadian physician and one of the four founding professors of Johns Hopkins Hospital

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Bernard Sachs (January 2, 1858 – February 8, 1944) was a Jewish-American neurologist

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Frederick Peterson (March 1, 1859 – July 9, 1938) was an American neurologist and poet. Peterson was at the forefront of psychoanalysis in the United States, publishing one of the first articles of Freud and Jung's theories of Free Association in 1909

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Sigmund Freud, born Sigismund Schlomo Freud, (6 May 1856 – 23 September 1939) was an Austrian neurologist and the father of psychoanalysis

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ETIOLOGY• Cerebral palsy is a heterogeneous disorder of

movement and posture that has a wide variety of presentations, ranging from mild motor disturbance to severe total body involvement

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ETIOLOGY• There are three distinctive features common to all

patients with cerebral palsy: • (1) some degree of motor impairment, versus autism; • (2) an insult to the developing brain, making it different

from conditions that affect the mature brain in older children and adults; and

• (3) a neurological deficit that is nonprogressive, versus other motor diseases of childhood, such as the muscular dystrophies

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ETIOLOGY• The insult to the brain is believed to occur between the

time of conception and age 2 years, at which time a significant amount of motor development has already occurred

• By 8 years of age, most of the development of the immature brain is complete, as is gait development, and an insult to the brain results in a more adult-type clinical picture and outcome

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ETIOLOGY• Although the neurological deficit is permanent and nonprogressive, the

effect it can have on the patient is dynamic, and the orthopaedic aspects of cerebral palsy can change dramatically with growth and development

• Growth, along with altered muscle forces across joints, can lead to (1)progressive loss of motion, (2)contracture, and eventually (3)joint subluxation or dislocation, resulting in (4)degeneration, that may require orthopaedic intervention

• http://www.restorativemedical.com/before_after_pics

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ETIOLOGY• Injury to the developing brain can occur at any

time from gestation to early childhood and typically is categorized as

1)Prenatal2)Perinatal3)Postnatal

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ETIOLOGY• Contrary to popular belief, fewer than 10% of injuries that result in cerebral

palsy occur during the birth process, with most occurring in the prenatal period

• A wide variety of risk factors for cerebral palsy have been identified in the prenatal period

1. This includes risk factors inherent to the fetus (most commonly genetic disorders)

2. Factors inherent to the mother (seizure disorders, mental retardation, and previous pregnancy loss)

3. Factors inherent to the pregnancy itself (Rh incompatibility, polyhydramnios, placental rupture, and drug or alcohol exposure)

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ETIOLOGY• External factors, such as TORCH syndrome

(toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex), also can lead to cerebral palsy in the prenatal period

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ETIOLOGY• Cerebral palsy in the perinatal period, from

birth until a few days after birth, typically is associated with asphyxia or trauma that occurs during labor

• Oxytocin augmentation, umbilical cord prolapse, and breech presentation all have been associated with an increased occurrence of cerebral palsy

• Only 10% of cases of cerebral palsy occur during this time period, and most patients with cerebral palsy have no history of asphyxia

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• https://www.google.co.in/imgres?imgurl=http://cdn3.nursingcrib.com/wp-content/uploads/prolapse-cord.jpg&imgrefurl=http://nursingcrib.com/nursing-notes-reviewer/maternal-child-health/umbilical-cord-prolapse/&h=320&w=400&tbnid=51zrYx8aV4NFpM:&docid=hDZBIIs16RimOM&ei=9gaIVuHmFoeVuATf2a2oDw&tbm=isch&ved=0ahUKEwjhufPh0YvKAhWHCo4KHd9sC_UQMwgxKAEwAQ

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ETIOLOGY• Although cerebral palsy often is associated with low

Apgar scores during this period, many neonates have low scores because of other conditions, such as genetic disorders, that are completely unrelated to asphyxia

• Low-birth-weight infants (<1500 g) are at dramatically increased risk of cerebral palsy, with an incidence of 60 per 1000 births compared with two per 1000 births in infants of normal weight

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ETIOLOGY• This increased incidence is believed to be due to the

fragility of the periventricular blood vessels, which are highly susceptible to physiological fluctuations during pregnancy

• These fluctuations, which include hypoxic episodes, placental pathology, maternal diabetes, and infection, can injure these vessels and lead to subsequent intraventricular hemorrhages

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ETIOLOGY• These injuries are graded on a scale from I to IV,

with an increased incidence of neurological consequences such as hydrocephalus and cerebral palsy in grade III (bleeding into ventricles with dilation) and grade IV (bleeding into brain substance)

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GRADING OF PERIVENTRICULAR LESIONS

I BLEEDING CONFINED TO GERMINAL MATRIX

II BLEEDING EXTENDS INTO VENTRICLES

III BLEEDING INTO VENTRICLES WITH DILATATION

IV BLEEDING INTO BRAIN SUBSTANCE

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A, In spastic diplegia and periventricular leukomalacia, the leg is more affected than the hand and face. There is no cortical injury

B, In spastic hemiplegia, the arm is often more affected than the leg. Because of cortical involvement, seizures and cognitive issues may occurhttp://clinicalgate.com/cerebral-palsy-4/

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ETIOLOGY• Hypoxic-ischemic encephalopathy, which is

characterized by hypotonia, decreased movement, and seizures, is a common cause of cerebral palsy during the postnatal period

• Meconium aspiration and persistent fetal circulation with true ischemia are the most common causes of hypoxic-ischemic encephalopathy

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CLASSIFICATION

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Classification• Because of the wide variability in presentation and types

of cerebral palsy, there is no universally accepted classification scheme

• Cerebral palsy can be classified by the (1) clinical physiological picture, the region of the body

affected, or (2) the neuroanatomical region of the brain that was injured

• It also can be classified (3) temporally in relation to the time of birth, as previously

described

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Geographical Classification• The anatomical region of the body affected with the

movement disorder should be identified • It is difficult to completely classify because some

extremities may be only subtly involved and a patient ’s pattern of involvement can change over time

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MONOPLEGIA HEMIPLEGIA PARAPLEGIA DIPLEGIA QUADRIPLEGIA DOUBLE HEMIPLEGIA TOTAL BODY

One extremity involved, usually lower

Very Rare

Both extremities on same side involvedUsually upper extremity involved more than lower extremity

30% patients

Both lower extremities equally involved

Very Rare

Lower extremities more involved than upper extremitiesFine-motor/sensory abnormalities in upper extremity

50% patients

All extremities involved equally

Normal head/neck control

All extremities involved, upper more than lower

All extremities severely involved

No head/neck control

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Monoplegia• Monoplegia is very rare and

usually occurs after meningitis • Most patients diagnosed with

monoplegia actually have hemiplegia with one extremity only very mildly affected

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Hemiplegia• In hemiplegia, one side of the

body is involved, with the upper extremity usually more affected than the lower extremity

• Patients with hemiplegia, approximately 30% of patients with cerebral palsy, typically have sensory changes in the affected extremities as well

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Hemiplegia• Severe sensory changes, especially in the upper

extremity, are a predictor of poor functional outcome after reconstructive surgery

• Hemiplegic patients also may have a leg-length discrepancy, with shortening on the affected side, which can be treated with contralateral epiphysiodesis or leg lengthening

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Diplegia• Diplegia is the most common

anatomical type of cerebral palsy, constituting approximately 50% of all cases

• Patients with diplegia have motor abnormalities in all four extremities, with the lower extremities more affected than the upper

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Diplegia• 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

• This type of cerebral palsy is most common in premature infants; intelligence usually is normal

• Most children with diplegia walk eventually, although walking is delayed usually until around age 4 years

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Quadriplegia• In quadriplegia, all four extremities are equally

involved and many patients have significant cognitive deficiencies that make care more difficult

• Head and neck control usually are present, which helps with communication, education, and seating

• Treatment goals for patients with quadriplegia include

(1)a straight spine and level pelvis, (2)located mobile hips with 90 degrees of flexion for

sitting and 30 degrees of extension for pivoting, (3)plantigrade feet that can fit in shoes, (4)and an appropriate wheelchair

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Total Body• Patients with total body involvement typically

have profound cognitive deficits in addition to loss of head and neck control

• These patients usually require full-time assistance for activities of daily living and specialized seating systems to assist with head positioning

• Drooling, dysarthria, and dysphagia also are common and complicate care

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Other Types• Some patients have a double

hemiplegia pattern as a result of bleeding in both hemispheres of the brain

• It often is difficult to differentiate this from diplegia or quadriplegia; however, in double hemiplegia, the upper extremities typically are more involved than the lower

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Other Types• Paraplegia is very rare and is

characterized by bilateral lower extremity involvement with—in contrast to diplegia — completely normal gross and fine motor skills in the upper extremity

• Many patients diagnosed with paraplegia actually are diplegic with very mildly involved upper extremities

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Other Types• Although occasionally mentioned, triplegia, the

involvement of three extremities, probably does not exist • With careful examination, most patients believed to have

triplegia actually have subtle motor deficits of the least involved limb

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Physiological Classification• Most patients with cerebral palsy have

recognizable patterns of movement that also can be classified

• An understanding of normal brain development is important

• During the first trimester, the immature brain separates into the gross structures, including the cerebrum, cerebellum, and medulla

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Physiological Classification• Neurons begin to form in the second trimester, and the

total number of neurons an individual eventually has are present at the end of this time frame

• Any neurons lost from this point forward are irreplaceable

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Physiological Classification• Synaptic connections and

myelination begin during the third trimester and continue through adolescence in a highly organized fashion

• As these synapses develop, and myelinization continues, primitive reflexes disappear and more mature motor patterns arise

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Physiological Classification• Because of this continued development after birth, many

injuries to the newborn nervous system go unrecognized until the absence of expected patterns can be detected

• Because different pathways of the brain are myelinated at different times,

1. spastic diplegia usually is not detected until 8 to 10 months of age;

2. hemiplegia, 20 months of age; 3. and athetoid cerebral palsy, after 24 months of age• It is important to keep this in mind because a child ’s

pattern may change over time42

Physiological Classification• Physiologically, cerebral palsy can be divided into a (1)spastic type, which affects the corticospinal (pyramidal) tracts, and (2)an extrapyramidal type, which affects the other regions of the

developing brain

• The extrapyramidal types of cerebral palsy include (1)athetoid A(2)choreiform C(3)ataxic X(4)rigid R(5)hypotonic H

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Spastic• Spastic is the most common form of cerebral

palsy, constituting approximately 80% of cases, and usually is associated with injury to the pyramidal tracts in the immature brain

• Spasticity, or the velocity-dependent increase in muscle tone with passive stretch, is caused by an exaggeration of the normal muscle passive stretch reflex

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Athetoid• Athetoid cerebral palsy 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; the results of soft tissue releases, in contrast to those seen in spastic cerebral palsy, are unpredictable, and the procedures have a high complication rate

• With the improvements in prevention of Rh incompatibility leading to kernicterus, the incidence of athetoid cerebral palsy is decreasing

• Dystonia, characterized by increased overall tone and distorted positioning in response to voluntary movements, or hypotonia also can occur with athetoid cerebral palsy

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Choreiform• Choreiform cerebral palsy is characterized by continual

purposeless movements of the patient ’ s wrists, fingers, toes, and ankles

• This continuous movement can make bracing and sitting difficult

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Ataxic• Ataxic cerebral palsy is very rare and probably is

the most often misdiagnosed type • It is characterized by the disturbance of

coordinated movement, most commonly walking, as a result of an injury to the developing cerebellum

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Rigid• Patients with rigid cerebral palsy are the most hypertonic

of all cerebral palsy patients • This hypertonicity occurs in the absence of hyperreflexia,

spasticity, and clonus, which are common in spastic cerebral palsy

• These patients have a “cogwheel” or “lead pipe” muscle stiffness that often requires surgical release

• When a surgical release is done, it is essential not to overweaken the muscle, which would cause the opposite deformity to occur

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Hypotonic• Hypotonic cerebral palsy is characterized by weakness

in conjunction with low muscle tone and normal deep tendon reflexes

• Many children who ultimately develop spastic or ataxic cerebral palsy pass through a hypotonic stage lasting 1 or 2 years before the true nature of their brain injury becomes apparent

• Persistent hypotonia can lead to difficulties with sitting balance, head positioning, and communication

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Mixed• Many patients with cerebral palsy have features of more

than one type and are referred to as having mixed cerebral palsy

• Patients with mixed cerebral palsy usually show signs of pyramidal and extrapyramidal deficits

• The final clinical appearance is determined by the relative components of spasticity, athetosis, and ataxia

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Mixed• Surgical releases in this group can be less predictable,

especially when a large athetoid or ataxic component is present

• Palisano et al. developed the Gross Motor Function Classification System to help resolve these classification difficulties

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LEVEL DESCRIPTION

1 Has nearly normal gross motor function

2Walks independently but has limitations with running and jumping

3Uses assistive devices to walk and wheelchair for long distances

4Has ability to stand for transfers but minimal walking ability; depends on wheelchair for mobility

5Lacks head control, cannot sit independently, and is dependent for all aspects of care

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Mixed• This five-level numeric grading system, which

has been found to be a reliable and stable method of classification and prediction of motor function for children 2 to 12 years old, takes into account functional limitations for assistive devices, such as walkers and wheelchairs, and the quality of movement based on age

• The emphasis of this scale is on self-initiated movement and walking and sitting function

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