Presentation on theme: "Cerebral Palsy Describes a group of disorders of movement and posture, limiting activity, attributed to non-progressive underlying brain pathology. The."— Presentation transcript:
1Cerebral PalsyDescribes a group of disorders of movement and posture, limiting activity, attributed to non-progressive underlying brain pathology.The motor disorders of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, or by a seizure disorder.
2Cerebral PalsyBrain lesions of CP occur from the fetal or neonatal period to up to age 3 yearsThe etiology of CP is not well understoodBrain lesions are thought to be associated with prenatal, perinatal, or postnatal events of varying causes.
3Cerebral Palsy Risk factors for CP are multifactorial. Birth, multiple gestation, intrauterine growth restriction, male sex, low APGAR scores, intrauterine infections, maternal thyroid abnormalities, prenatal strokes, birth asphyxia, maternal methyl mercury exposure, and maternal iodine deficiency
4Cerebral Palsy Prevalence In developed countries: about cases per 1000 live birthsIn developing countries: about cases per 1000 live births.
6General Clinical Presentations of Cerebral Palsy Failure to meet expected developmental milestones or failing to suppress obligatory primitive reflexes.Abnormalities in muscle tone. Presents as either hypotonic or hypertonic with either decreased or increased resistance to passive movements, respectively.Definite hand preference before age 1 year is a red flag for possible hemiplegia.Asymmetric crawling or failure to crawl also may suggest cerebral palsy.
7General Clinical Presentations of Cerebral Palsy Joint contractures secondary to spastic musclesHypotonic to spastic toneGrowth delayPersistent primitive reflexesGait pattern abnormalitiesHip - Excessive flexion, adduction, and femoral anteversion Knee - Flexion and extension with valgus or varus stress occur.Foot - Equinus, or toe walking, and varus or valgus of the hindfoot
8Types of Cerebral Palsy Spastic (70-80%) – Increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife responseSpastic diplegia (30-40%) – lower extremity involvementSpastic hemiplegia (20-30%) – 1 side of the body involvedSpastic quadriplegia (10-15%) – total body involvementSpastic monoplegia (rare) – 1 limb involved
9Types of Cerebral Palsy Dyskinetic (10-15%) – Fluctuating tone, rigid total body involvement by definition. Persistent primitive reflex patterns (asymmetric tonic neck reflex, labyrinthine)Athetoid – slow writhing movementsDystonic – posturing of the head, trunk, and extremities
10Types of Cerebral Palsy Ataxic (<5%) – characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement.
11Types of Cerebral Palsy Mixed - no single specific tonal quality predominating; mixture of spastic and dyskinetic componentsHypotonic - truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare
13Associated Conditions Cognitive and linguistic:Mental retardationHigh incidence of language and learning disabilitiesDysarthriaAttention deficit hyperactivity disorderSleep and behavioral disturbances
16Spastic Hemiplegic CP One-sided upper motor neuron deficit Arm generally affected more than leg; possible early hand preference or relative weakness on one sideGait characterized by circumduction of lower extremity on affected sideSpecific learning disabilitiesOromotor dysfunctionPossible unilateral sensory deficitsVisual-field deficits (eg, homonymous hemianopsia) and strabismusSeizures
17Spastic Diplegic CPUpper motor neuron findings in the legs more than the arms. Little or no functional limitation of the upper extremities.Scissoring gait pattern with hips flexed and adducted, knees flexed with valgus, and ankles in equinus, resulting in toe walkingDelay in developing gross motor skills.
18Spastic Quadriplegic CP All limbs affected, either full-body hypertonia or truncal hypotonia with extremity hypertoniaOromotor dysfunctionIncreased risk of cognitive difficultiesMultiple medical complicationsSeizuresLegs generally affected equally or more than arms
19Dyskinetic CPEarly hypotonia with movement disorder emerging at age 1-3 yearsArms more affected than legsDeep tendon reflexes usually normal to slightly increasedSome spasticityOromotor dysfunctionGait difficultiesTruncal instabilityRisk of deafness in those affected by kernicterus
20Ataxic CP Hypotonic Tremors Motor skills might be affected( i.e. writing, typing, or using scissors)Difficulty in balance esp. while walkingDifficulty with visual and/or auditory processing
22Pathophysiology<10% children with CP : evidence of intrapartum asphyxiaAssociated with increased risk of CP in normal birthweight infants: intrauterine exposure to maternal infectionChorioamnionitisInflammation of the placental membranes and umbilical cordFoul smelling amniotic fluidMaternal sepsisMaternal temp greater than 38⁰C during laborUTI
23PathophysiologyPrevalence of CP is increased among low birthweight infants, particularly those weighing <1,000 g at birth : because of intracerebral haemorrhage and periventricular leukomalacia (PVL)PVL: appears to reflect the enhanced vulnerability of immature oligodendroglia in premature infants to oxidative stress caused by ischemia or infectious/inflammatory insults
24PathophysiologyBelieved to be caused by nonprogressive disturbances in the immature and still developing fetal or infant brainDisturbances affect the development of movement and posture but patients are also frequently seen to have epilepsy, secondary musculoskeletal problems and sensation, perception, cognition, communication and behavior disturbances.
25PathophysiologyInsult to immature brain (before birth to postnatal period) if immediately after postnatal period may be due to hypoxic-ischemic encephalopathy cerebral insult altered muscle tone, muscle stretch reflexes, primitive reflexes, postural ractionsCerebral insults may be vascular, hypoxic-ischemic, metabolic, infectious, toxic, teratogenic, traumatic, and genetic in nature
26Classification of Cerebral Palsy and Major Causes (Nelsons 17th Ed.) MOTOR SYNDROMENEUROPATHYMAJOR CAUSESSpastic DiplegiaPeriventricularLeukomalacia (periventricular leukomlacic [PVL])PrematurityIschemiaInfectionEndocrine/metabolic (e.g., thyroid)Spastic QuadriplegiaPVLMulticystic encephalomalaciaMalformationsEndocrine/metabolicGenetic/developmentalHemiplegiaStoke: in utero or neonatalThrombophilic disordersPeriventricular hemorrhagic infearctionExtrapyramidal (athetoid, dyskenetic)Basal gangliaPathology: putamen, globus pallidus, thalamusAsphyxiaKernicterusMitochondrialGenetic/metabolic
27Pathophysiology Spastic Hemiplegia focal cerebral infarction secondary to intrauterine or perinatal thromboembolism related to thrombophilic disorders, especially anticardiolipin antibodies, is an important causefamily histories suggesting thrombosis and inherited clotting disorders may be present
28Pathophysiology Spastic Diplegia Spastic Quadriplegia the most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsuleSpastic Quadriplegiamost severe form of CP; swallowing difficulties are common as a result of supranuclear bulbar palsies, often leading to aspirationthe most common lesions seen are severe PVL and multicystic cortical encephalomalacia
29Pathophysiology Athetoid/Chorioathetoid/Extraoyramidal CP if secondary to acute intrapartum near-total asphyxia is associated with bilateral symmetric lesions in the posterior putamen and ventrolateral thalamuscan also be caused by kernicterus secondary to high levels of bilirubincan also be associated with lesions in the basal ganglia and thalamus caused by metabolic genetic disorders such as mitochondrial disorders and glutaric aciduria
31Therapy Goal: to maximize the functional use of limbs and ambulation to reduce the risk of contracturesto help the patient in attaining his greatest potential physically, mentally and socially
32RehabilitationPhysical therapy – to develop muscle strength, flexibility and strengthOccupational therapy – to help learn physical skills needed to function in everyday lifeRecreational therapyOrthotic devices such as ankle foot orthosesSpeech therapy – to overcome speech problemsPsychotherapy
33Parent educationTeach the parents how to work with their child in daily activities such as feeding, dressing, bathing, and playing in ways that limit the effects of abnormal muscle tone.Instruct the parents in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon.
34For children with Spastic Diplegia Use walkers, poles, and standing framesSurgery may be considered to reduce muscle spasm around the hip girdle (adductor tenotomy or psoas transfer and release)Rhizotomy procedure – roots of the spinal nerves are divided, produces considerable improvement in some patientsConsider surgery if pt has marked spasticity of the lower extremities or evidence of hip dislocationTenotomy is the cutting of a tendon. It is performed in order to lengthen a muscle that has developed improperly, or become shortened and is resistant to stretch. Read more: Tenotomy - procedure, pain, time, infection, operation, medication, risk, children, rate, Definition, Purpose, Demographics, Description, Diagnosis/Preparation, Aftercare, Risks, Normal results34
35For children with Spastic Hemiplegia A tight heel cord may be treated by tenotomy of the Achilles tendonConstraints can be applied to the unaffected side – this induces improved hand and arm functioning on the affected side. This is effective in patients of all ages.
36For children with Spastic Quadriplegia Use motorized wheelchairs, special feeding devices, modified typewriters, and customized seating arrangements
37Surgical To correct anatomical abnormalities or release tight muscles To help repair dislocated hips and scoliosis (curvature of the spine)
38Surgery Dorsal rhizotomy (for severe spastic diplegia) Cut specific nerves at their roots to reduce spasticityStereotactic surgeryTo improve rigidity, athetosis and tremorsReconstructive surgery to an armto restore muscle balance, release contractures, and stabilize joints
39Medical TherapyGoal of pharmacotherapy is to reduce symptoms (e.g. spasticity) and prevent complications (e.g. contractures)2 types of medicationsFor spasticity and abnormal movementFor seizures
40Medications for spasticity Dopaminergic drugsincrease dopamine levels to decrease rigidity and abnormal movementsE.g. levodopa/carbidopaBotulinum toxinInjected into specific muscle groupsShows very positive responseMay also be used to reduce the severity of drooling when injected into the salivary glands
41Medications for spasticity Muscle relaxantsBaclofen: controls muscle contractions and relaxes tight muscles, but lowers seizure thresholdBotulinium toxin A: causes mild muscle paralysis and reduce contractionsBenzodiazepines (valium) – sedation is a side-effectOral dantrolene sodiumNeed constant follow-up
42Medications for seizures AnticonvulsantsUsed to terminate clinical and electrical seizure activity as rapidly as possiblePrevent seizure recurrencePhenobarbital or phenytoinEffective against partial seizuresBenzodiazepinesUsed in acute management of seizures
43Other ProblemsImportant to identify and manage behavioral problems early - work with a psychologist or psychiatristLearning and attention deficit disorders, and mental retardation – assessed and managed by a psychologist and educatorStrabismus, nystagmus, and optic atrophy are common – consult an ophthalmologistPromptly assess and treat lower urinary tract dysfunctionCommunication - Use Blissymbolics, talking typewriters, and specially adapted computersBlissymbols or Blissymbolics were conceived of as an ideographic writing system consisting of several hundred basic symbols, each representing a concept, which can be composed together to generate new symbols that represent new concepts. Blissymbols differ from all the world's major writing systems in that the characters do not correspond at all to the sounds of any spoken language.However, since the 1960s, Blissymbols have become popular as a method of augmentative and alternative communication (AAC) for non-speaking people with cerebral palsy or other disorders, for whom it can be impossible to otherwise communicate with spoken language. Practitioners of Blissymbolics (that is, speech and language therapists and users) maintain that some users who have learned to communicate with Blissymbolics find it easier to learn to read and write traditional orthography in the local spoken language than do users who did not know Blissymbolics.43
44Reference Nelson’s Textbook of Pediatrics 18th ed.