cerebral palsy the abc's of cp

Cerebral PalsyCerebral PalsyThe ABC’s The ABC’s of CPof CP

Toni Benton, M.D.Toni Benton, M.D.Continuum of Care ProjectContinuum of Care Project

UNM HSC School of MedicineUNM HSC School of MedicineApril 20, 2006April 20, 2006

Cerebral PalsyCerebral Palsy

OutlineOutlineI. DefinitionI. DefinitionII. Incidence, Epidemiology and DistributionII. Incidence, Epidemiology and DistributionIII. EtiologyIII. EtiologyIV. TypesIV. TypesV. Medical ManagementV. Medical ManagementVI. Psychosocial IssuesVI. Psychosocial IssuesVII. AgingVII. Aging

Cerebral Palsy-Cerebral Palsy-DefinitionDefinition Cerebral palsy is a symptom complex, (not a disease) that has Cerebral palsy is a symptom complex, (not a disease) that has

multiple etiologies.multiple etiologies.

CP is a disorder of tone, posture or movement due to a lesion in CP is a disorder of tone, posture or movement due to a lesion in the developing brain.the developing brain.

Lesion results in paralysis, weakness, incoordination or abnormal Lesion results in paralysis, weakness, incoordination or abnormal movementmovement

Not contagious, no cure.Not contagious, no cure.

It is static, but it symptoms may change with maturationIt is static, but it symptoms may change with maturation


Brain damageBrain damage

Occurs during developmental periodOccurs during developmental period

Motor dysfunctionMotor dysfunction

Not CurableNot Curable

Non-progressive (static)Non-progressive (static)Any regression or deterioration of motor or Any regression or deterioration of motor or intellectual skills should prompt a search for a intellectual skills should prompt a search for a degenerative diseasedegenerative disease

Therapy can help improve functionTherapy can help improve function


There are 2 major types of CP, There are 2 major types of CP, depending on location of lesions:depending on location of lesions: Pyramidal (Spastic)Pyramidal (Spastic) ExtrapyramidalExtrapyramidal

There is overlap of both symptoms There is overlap of both symptoms and anatomic lesions.and anatomic lesions.

The pyramidal system carries the The pyramidal system carries the signal for muscle contraction.signal for muscle contraction.

The extrapyramidal system provides The extrapyramidal system provides regulatory influences on that regulatory influences on that contraction.contraction.


Types of brain damageTypes of brain damage BleedingBleeding Brain malformationBrain malformation Trauma to brainTrauma to brain Lack of oxygenLack of oxygen InfectionInfection ToxinsToxins UnknownUnknown

EpidemiologyEpidemiology

The overall prevalence of cerebral palsy ranges The overall prevalence of cerebral palsy ranges from 1.5 to 2.5 per 1000 live births.from 1.5 to 2.5 per 1000 live births.

The overall prevalence of CP has remained stable The overall prevalence of CP has remained stable since the 1960’s.since the 1960’s.

Speculations that the increased survival of the Speculations that the increased survival of the VLBW preemies would cause a rise in the VLBW preemies would cause a rise in the prevalence of CP have proven wrong.prevalence of CP have proven wrong.

Likewise the expected decrease in CP as a result of Likewise the expected decrease in CP as a result of C-section and fetal monitoring has not happened.C-section and fetal monitoring has not happened. However, the prevalence of the subtypes has However, the prevalence of the subtypes has

changed.changed.

EpidemiologyEpidemiology

Due to the increased survival of very Due to the increased survival of very low birth weight preemies, the low birth weight preemies, the incidence of spastic diplegia has incidence of spastic diplegia has increased.increased.

Choreoathetoid CP, due to Choreoathetoid CP, due to kernicterus, has decreased.kernicterus, has decreased.

Multiple gestation carries an Multiple gestation carries an increased risk of CP. increased risk of CP.

Distribution of the Types of Distribution of the Types of CPCPTypes of Types of

Cerebral PalsyCerebral PalsyFrequency of Frequency of DistributionDistribution

Nonspastic Nonspastic (extrapyramidal and (extrapyramidal and mixed types)mixed types)

23%23%

Spastic CP (total)Spastic CP (total) 77%77%

Spastic DiplegiaSpastic Diplegia 21%21%

Spastic HemiplegiaSpastic Hemiplegia 21%21%

Spastic QuadriplegiaSpastic Quadriplegia 23%23%

EtiologyEtiology

CP has multiple etiologies- many are CP has multiple etiologies- many are still unknownstill unknown

Since CP is not a single entity, Since CP is not a single entity, recurrence risks depend on the recurrence risks depend on the underlying cause.underlying cause.

If there is a regression in skills, If there is a regression in skills, suspect a degenerative disease.suspect a degenerative disease.

EtiologyEtiology

Most causes are Most causes are preprenatal- genetic, natal- genetic, congenital malformations, metabolic, congenital malformations, metabolic, intrauterine infections, rather than intrauterine infections, rather than periperinatal or natal or postpostnatal- birth natal- birth asphyxia, hemorrhage, infarction, asphyxia, hemorrhage, infarction, infections, trauma.infections, trauma.

EtiologyEtiology

Much of the literature of the 1990’s was Much of the literature of the 1990’s was directed at the controversy re the role of directed at the controversy re the role of asphyxia in the etiology of CPasphyxia in the etiology of CP Asphyxia implies poor gas exchange, low Asphyxia implies poor gas exchange, low

Apgars and neurologic depression during and Apgars and neurologic depression during and soon after delivery. soon after delivery.

Significant asphyxia is accompanied by Significant asphyxia is accompanied by acidosis.acidosis.

Asphyxia is rarely the cause of CP in the term Asphyxia is rarely the cause of CP in the term infant.infant.

EtiologyEtiology

In one outcome study of 43,437 In one outcome study of 43,437 full termfull term children, 150 had cerebral palsy. Only 9 of children, 150 had cerebral palsy. Only 9 of these cases were attributable to birth these cases were attributable to birth asphyxia. asphyxia.

34 had spastic quadriplegia and 71% of 34 had spastic quadriplegia and 71% of those cases had identifiable causes.those cases had identifiable causes.

53%- congenital disorders53%- congenital disorders14%-birth asphyxia14%-birth asphyxia8%-CNS infections8%-CNS infections

EtiologyEtiology

Among the children with non Among the children with non quadriplegic cerebral palsy, quadriplegic cerebral palsy, congenital disorders appeared to congenital disorders appeared to account for about 1/3 of the cases, account for about 1/3 of the cases, and CNS infections accounted for 5%.and CNS infections accounted for 5%.

(Wilson and Cooley-2000; Collaborative Perinatal Study of The National Institute of (Wilson and Cooley-2000; Collaborative Perinatal Study of The National Institute of Neurological and Communicative Disorders and Stroke,Naeye, 1989)Neurological and Communicative Disorders and Stroke,Naeye, 1989)

Hypoxic Ischemic Hypoxic Ischemic Encephalopathy (HIE)Encephalopathy (HIE)

A clinical entity first described in 1976A clinical entity first described in 1976 Used interchangeably with Neonatal Used interchangeably with Neonatal

encephalopathy.encephalopathy. Asphyxia refers to the first minutes Asphyxia refers to the first minutes

after birth (low Apgars and acidosis)after birth (low Apgars and acidosis) HIE signs and symptoms persist over HIE signs and symptoms persist over

hours and days that follow.hours and days that follow.

Hypoxic Ischemic Hypoxic Ischemic Encephalopathy (HIE)Encephalopathy (HIE)

3 major lesions arise from HIE3 major lesions arise from HIE1.1. Periventricular Leukomalacia (PVL) Periventricular Leukomalacia (PVL)

Typically seen in the premature infantTypically seen in the premature infant

a. Hemorrhagic PVLa. Hemorrhagic PVL

b. Ischemic PVLb. Ischemic PVL

2.2. Parasaggital Cerebral InjuryParasaggital Cerebral Injury Typically seen in the term infantTypically seen in the term infant

3.3. Selective (Focal) Neuronal NecrosisSelective (Focal) Neuronal Necrosis Seen in both term and premature infantsSeen in both term and premature infants

Periventricular Leukomalacia Periventricular Leukomalacia (PVL)(PVL)

1.1. Hemorrhagic PVLHemorrhagic PVL• Hemorrhage is associated with a collection of Hemorrhage is associated with a collection of

primitive cells between the ependyma and primitive cells between the ependyma and caudate that are programmed to “melt away” caudate that are programmed to “melt away” at 32-34 weeks gestationat 32-34 weeks gestation

• They contain fragile capillaries that are easily They contain fragile capillaries that are easily damaged by hypoxia (lack of oxygen) and damaged by hypoxia (lack of oxygen) and hypotension (drop in blood pressure).hypotension (drop in blood pressure).

• When the blood pressure returns to normal, When the blood pressure returns to normal, bleeding occurs because the preemie has bleeding occurs because the preemie has underdeveloped autoregulation.underdeveloped autoregulation.


1.1. Hemorrhagic PVLHemorrhagic PVL (cont.)(cont.)• This bleeding may then rupture into This bleeding may then rupture into

the ventricle and/or parenchymathe ventricle and/or parenchyma• Periventricular venous congestion Periventricular venous congestion

(swelling) may then occur, and cause (swelling) may then occur, and cause ischemia (lack of blood supply) and ischemia (lack of blood supply) and periventricular hemorrhagic infarction. periventricular hemorrhagic infarction.

Periventricular Leukomalacia Periventricular Leukomalacia (PVL)(PVL)2. 2. Ischemic PVLIschemic PVL

An ischemic infarction or failure of An ischemic infarction or failure of perfusion usually to the watershed area perfusion usually to the watershed area surrounding the ventricular horns- “HIE surrounding the ventricular horns- “HIE white matter necrosis”.white matter necrosis”.

Peak incidence occurs around 32 weeksPeak incidence occurs around 32 weeks Larger infarcts may leave a cyst Larger infarcts may leave a cyst Secondary hemorrhage can occur into Secondary hemorrhage can occur into

theses cysts- “periventricular theses cysts- “periventricular hemorrhage”.hemorrhage”.

Periventricular leukomalaciaPeriventricular leukomalacia


2. Ischemic PVL2. Ischemic PVL PVL can extend into the internal PVL can extend into the internal

capsule and result in hemiplegia capsule and result in hemiplegia superimposed on diplegia. superimposed on diplegia.

Prenatal maternal ultrasound has Prenatal maternal ultrasound has detected lesions in the fetus at 28-32 detected lesions in the fetus at 28-32 weeks gestation, thus confirming weeks gestation, thus confirming that PVL can occur prenatally.that PVL can occur prenatally.

Internal CapsuleInternal Capsule

Parasaggital Cerebral InjuryParasaggital Cerebral Injury

Injury is related to vascular factors, Injury is related to vascular factors, especially in the parasaggital border zones especially in the parasaggital border zones that are more vulnerable to a drop in that are more vulnerable to a drop in perfusion pressure and immature perfusion pressure and immature autoregulation.autoregulation.

The ischemic lesion results in cortical and The ischemic lesion results in cortical and subcortical white matter injury.subcortical white matter injury.

It is usually bilateral and symmetric.It is usually bilateral and symmetric. The posterior aspect of the cerebral The posterior aspect of the cerebral

hemisphere especially the parietal occipital hemisphere especially the parietal occipital regions is more affected than the anterior.regions is more affected than the anterior.

Selective (Focal) Neuronal Selective (Focal) Neuronal Necrosis (SNN)Necrosis (SNN)

Occurs in the glutamate sensitive areas in Occurs in the glutamate sensitive areas in the basal ganglia, thalamus, brainstem and the basal ganglia, thalamus, brainstem and cortex.cortex.

The location of the focal necrosis, which The location of the focal necrosis, which show up as cystic lesions on MRI, depend on show up as cystic lesions on MRI, depend on the stage of development of the infant’s the stage of development of the infant’s brain at the time of the HIE.brain at the time of the HIE. For example, HIE at term often produces SNN in For example, HIE at term often produces SNN in

the basal ganglia since it is glutamate sensitive the basal ganglia since it is glutamate sensitive and very hypermetabolic at term. and very hypermetabolic at term.

Types of Cerebral PalsyTypes of Cerebral Palsy

PyramidalPyramidal Described as a Clasped Described as a Clasped

knife response orknife response or Velocity dependent Velocity dependent

increased resistance to increased resistance to passive muscle stretchpassive muscle stretch

The spasticity can be The spasticity can be worse when the person worse when the person is anxious or ill.is anxious or ill.

The spasticity does not The spasticity does not go away when the go away when the person is asleep.person is asleep.

ExtrapyramidalExtrapyramidal AtaxiaAtaxia HypotoniaHypotonia DystoniaDystonia Rigidity Rigidity

The tone may increase The tone may increase with volitional with volitional movement, or when the movement, or when the person is anxiousperson is anxious

During sleep the person During sleep the person is actually hypotonicis actually hypotonic

Anatomy of motor lesions- Anatomy of motor lesions- pyramidal systempyramidal system

Types of Cerebral PalsyTypes of Cerebral Palsy

A.A. Pyramidal (Spastic)Pyramidal (Spastic)• Quadriplegia- all 4 extremitiesQuadriplegia- all 4 extremities• Hemiplegia- one side of the bodyHemiplegia- one side of the body• Diplegia- legs worse than armsDiplegia- legs worse than arms• Paraplegia- legs onlyParaplegia- legs only• Monoplegia- one extremityMonoplegia- one extremity

B. ExtrapyramidalB. Extrapyramidal

Divided into Dyskinetic and Ataxic typesDivided into Dyskinetic and Ataxic types

DyskineticDyskinetic Athetosis- slow Athetosis- slow

writhing, wormlikewrithing, wormlike Chorea- quick, jerky Chorea- quick, jerky

movementsmovements Choreoathetosis- Choreoathetosis-

mixedmixed Hypotonia- floppy, low Hypotonia- floppy, low

muscle tone, little muscle tone, little movementmovement

Ataxic CPAtaxic CP Results from damage Results from damage

to the cerebellumto the cerebellum Ataxia- tremor & Ataxia- tremor &

drunken- like gaitdrunken- like gait

AnatomyAnatomy

PyramidalPyramidal Lesion is usually in Lesion is usually in

the motor cortex, the motor cortex, internal capsule internal capsule and/or cortical and/or cortical spinal tracts.spinal tracts.

ExtrapyramidalExtrapyramidal Lesion is usually in Lesion is usually in

the basal ganglia, the basal ganglia, Thalamus, Thalamus, Subthalamic Subthalamic nucleus and/or nucleus and/or cerebellum.cerebellum.

Comparison of SymptomsComparison of SymptomsPyramidalPyramidal ExtrapyramidaExtrapyramida

ll

ToneTone increasedincreased alternatingalternating

Type of toneType of tone spasticspastic rigidrigid

DTR’sDTR’s increasedincreased normal to normal to increasedincreased

ClonusClonus PresentPresent occ. presentocc. present

ContracturesContractures earlyearly latelate

Primitive Primitive ReflexesReflexes

delayeddelayed persistentpersistent

Involuntary Involuntary movementsmovements

rarerare frequentfrequent

Medical ManagementMedical Management

GrowthGrowth Persons with CP often have struggle to Persons with CP often have struggle to

gain or maintain weight. gain or maintain weight. Failure to Thrive is a common problem.Failure to Thrive is a common problem.

Before diagnosing Failure to thrive, an accurate Before diagnosing Failure to thrive, an accurate Body Mass Index must be obtained, but an Body Mass Index must be obtained, but an accurate height is difficult to obtain in a person accurate height is difficult to obtain in a person with severe contractures.with severe contractures.

In such cases, arm span calculations may be In such cases, arm span calculations may be used and a growth chart is available to used and a growth chart is available to determine percentiles standardized to age and determine percentiles standardized to age and gender.gender.

Extremity length growth Extremity length growth chartchart


Orthopedic ProblemsOrthopedic Problems

ScoliosisScoliosis Hip DislocationsHip Dislocations ContracturesContractures OsteoporosisOsteoporosis


Oromotor DysfunctionOromotor Dysfunction Especially common in persons with Especially common in persons with

Extrapyramidal CP and Spastic Extrapyramidal CP and Spastic quadriplegiaquadriplegia Language delay/Speech delaysLanguage delay/Speech delays DroolingDrooling DysphagiaDysphagia AspirationAspiration


Gastrointestinal DysmotilityGastrointestinal Dysmotility Delayed gastric emptyingDelayed gastric emptying Gastroesophageal refluxGastroesophageal reflux

PainPain Chronic aspirationChronic aspiration

ConstipationConstipation

These disorders are interrelated and These disorders are interrelated and compound one another.compound one another.


Spasticity ManagementSpasticity ManagementManagement of spasticity does not fix the Management of spasticity does not fix the

underlying pathology of CP, but it may underlying pathology of CP, but it may decreased the sequelae of increased tone.decreased the sequelae of increased tone.

Over time, the spasticity leads to:Over time, the spasticity leads to: musculoskeletal deformity musculoskeletal deformity

scoliosis scoliosis hip dislocation hip dislocation contracturescontractures

PainPain Hygiene problemsHygiene problems

Treatment of SpasticityTreatment of Spasticity

MedicationsMedications ValiumValium DantriumDantrium BaclofenBaclofen ClonidineClonidine ClonazepamClonazepam BOTOXBOTOX

Treatment of DystoniaTreatment of Dystonia

Medications-(None are very effective)Medications-(None are very effective) L-Dopa- drug of choice for certain disordersL-Dopa- drug of choice for certain disorders ArtaneArtane Anticonvulsants-for intermittent and paroxysmal Anticonvulsants-for intermittent and paroxysmal

dystoniadystonia Anti-spasticity medications-Anti-spasticity medications- Haldol or Reserpine- for choreoathetosisHaldol or Reserpine- for choreoathetosis Propranolol- for essential tremorPropranolol- for essential tremor Clonazepam or Valium- for “rubral tremors”-Clonazepam or Valium- for “rubral tremors”-

(course tremors of the entire arm)(course tremors of the entire arm) Valproic acid or clonazepam for action myoclonus- Valproic acid or clonazepam for action myoclonus-

(large jerks with intentional movements)(large jerks with intentional movements)

Associated ProblemsAssociated Problems

Mental RetardationMental Retardation Communication Communication

DisordersDisorders NeurobehavioralNeurobehavioral SeizuresSeizures Vision DisordersVision Disorders Hearing lossHearing loss Somatosensation Somatosensation

(skin sensation, body (skin sensation, body awareness)awareness)

Temperature instabilityTemperature instability NutritionNutrition DroolingDrooling Dentition problemsDentition problems Neurogenic bladderNeurogenic bladder Neurogenic bowelNeurogenic bowel Gastroesophageal Gastroesophageal

refluxreflux DysphagiaDysphagia Autonomic dysfunctionAutonomic dysfunction

Other TreatmentsOther Treatments

CastingCasting Therapeutic Electrical StimulationTherapeutic Electrical Stimulation Patterning: Doman-Delacato- Patterning: Doman-Delacato- (not (not

recommended) recommended) Selective Dorsal RhizotomySelective Dorsal Rhizotomy MassageMassage Hyperbaric OxygenHyperbaric Oxygen AcupunctureAcupuncture

Adult ConcernsAdult Concerns

MedicalMedical Routine Healthcare MaintenanceRoutine Healthcare Maintenance Sequelae of SpasticitySequelae of Spasticity Orthopedic IssuesOrthopedic Issues Pain ManagementPain Management Neurogenic Bowel and BladderNeurogenic Bowel and Bladder Prevention of Chronic Aspiration Management Prevention of Chronic Aspiration Management

of Gastroesophageal Reflux & Complicationsof Gastroesophageal Reflux & Complications Barrett’s esophagusBarrett’s esophagus Esophageal stricturesEsophageal strictures Esophageal/stomach cancerEsophageal/stomach cancer

Adult ConcernsAdult Concerns

PsychosocialPsychosocial

Transition from Pediatric to Adult servicesTransition from Pediatric to Adult servicesIndependenceIndependence

WorkWorkHomeHome

RelationshipsRelationshipsGuardianshipGuardianshipEnd of lifeEnd of life

cerebral palsy the abc's of cp

Documents

etiology cp

etiology of cp asphyxia

cause of cp

quadriplegic cerebral

cerebral palsy outline

cerebral palsy brain

major types of cp

spastic cp total