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:
Cerebral Palsy Describes 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.
Cerebral Palsy Brain lesions of CP occur from the fetal or neonatal period to up to age 3 years The etiology of CP is not well understood Brain lesions are thought to be associated with prenatal, perinatal, or postnatal events of varying causes.
Cerebral 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
Cerebral Palsy Prevalence – In developed countries: about cases per 1000 live births – In developing countries: about cases per 1000 live births.
Clinical Presentations of Cerebral Palsy
General 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.
General Clinical Presentations of Cerebral Palsy Joint contractures secondary to spastic muscles Hypotonic to spastic tone Growth delay Persistent primitive reflexes Gait pattern abnormalities – Hip - 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
Types of Cerebral Palsy Spastic (70-80%) – Increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response – Spastic diplegia (30-40%) – lower extremity involvement – Spastic hemiplegia (20-30%) – 1 side of the body involved – Spastic quadriplegia (10-15%) – total body involvement – Spastic monoplegia (rare) – 1 limb involved
Types 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 movements – Dystonic – posturing of the head, trunk, and extremities
Types of Cerebral Palsy Ataxic (<5%) – characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement.
Types of Cerebral Palsy Mixed - no single specific tonal quality predominating; mixture of spastic and dyskinetic components Hypotonic - truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare
Associated Conditions Cognitive and linguistic: – Mental retardation – High incidence of language and learning disabilities – Dysarthria – Attention deficit hyperactivity disorder – Sleep and behavioral disturbances
Spastic Hemiplegic CP One-sided upper motor neuron deficit Arm generally affected more than leg; possible early hand preference or relative weakness on one side Gait characterized by circumduction of lower extremity on affected side Specific learning disabilities Oromotor dysfunction Possible unilateral sensory deficits Visual-field deficits (eg, homonymous hemianopsia) and strabismus Seizures
Spastic Diplegic CP Upper 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 walking Delay in developing gross motor skills.
Spastic Quadriplegic CP All limbs affected, either full-body hypertonia or truncal hypotonia with extremity hypertonia Oromotor dysfunction Increased risk of cognitive difficulties Multiple medical complications Seizures Legs generally affected equally or more than arms
Dyskinetic CP Early hypotonia with movement disorder emerging at age 1-3 years Arms more affected than legs Deep tendon reflexes usually normal to slightly increased Some spasticity Oromotor dysfunction Gait difficulties Truncal instability Risk of deafness in those affected by kernicterus
Ataxic CP Hypotonic Tremors Motor skills might be affected( i.e. writing, typing, or using scissors) Difficulty in balance esp. while walking Difficulty with visual and/or auditory processing
Pathophysiology of Cerebral Palsy
Pathophysiology <10% children with CP : evidence of intrapartum asphyxia Associated with increased risk of CP in normal birthweight infants: intrauterine exposure to maternal infection – Chorioamnionitis – Inflammation of the placental membranes and umbilical cord – Foul smelling amniotic fluid – Maternal sepsis – Maternal temp greater than 38⁰C during labor – UTI
Pathophysiology Prevalence 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
Pathophysiology Believed to be caused by nonprogressive disturbances in the immature and still developing fetal or infant brain Disturbances 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.
Pathophysiology Insult 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 ractions Cerebral insults may be vascular, hypoxic-ischemic, metabolic, infectious, toxic, teratogenic, traumatic, and genetic in nature
Classification of Cerebral Palsy and Major Causes (Nelsons 17th Ed.) MOTOR SYNDROMENEUROPATHYMAJOR CAUSES Spastic DiplegiaPeriventricular Leukomalacia (periventricular leukomlacic [PVL]) Prematurity Ischemia Infection Endocrine/metabolic (e.g., thyroid) Spastic QuadriplegiaPVL Multicystic encephalomalacia Malformations Ischemia Infection Endocrine/metabolic Genetic/developmental HemiplegiaStoke: in utero or neonatalThrombophilic disorders Infection Genetic/developmental Periventricular hemorrhagic infearction Extrapyramidal (athetoid, dyskenetic) Basal ganglia Pathology: putamen, globus pallidus, thalamus Asphyxia Kernicterus Mitochondrial Genetic/metabolic
Pathophysiology Spastic Hemiplegia – focal cerebral infarction secondary to intrauterine or perinatal thromboembolism related to thrombophilic disorders, especially anticardiolipin antibodies, is an important cause – family histories suggesting thrombosis and inherited clotting disorders may be present
Pathophysiology Spastic Diplegia – the most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule Spastic Quadriplegia – most severe form of CP; swallowing difficulties are common as a result of supranuclear bulbar palsies, often leading to aspiration – the most common lesions seen are severe PVL and multicystic cortical encephalomalacia
Pathophysiology Athetoid/Chorioathetoid/Extraoyramidal CP – if secondary to acute intrapartum near-total asphyxia is associated with bilateral symmetric lesions in the posterior putamen and ventrolateral thalamus – can also be caused by kernicterus secondary to high levels of bilirubin – can also be associated with lesions in the basal ganglia and thalamus caused by metabolic genetic disorders such as mitochondrial disorders and glutaric aciduria
Therapy Goal: – to maximize the functional use of limbs and ambulation – to reduce the risk of contractures – to help the patient in attaining his greatest potential physically, mentally and socially
Rehabilitation Physical therapy – to develop muscle strength, flexibility and strength Occupational therapy – to help learn physical skills needed to function in everyday life Recreational therapy Orthotic devices such as ankle foot orthoses Speech therapy – to overcome speech problems Psychotherapy
Parent education Teach 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.
For children with Spastic Diplegia Use walkers, poles, and standing frames Surgery 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 patients
For children with Spastic Hemiplegia A tight heel cord may be treated by tenotomy of the Achilles tendon Constraints 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.
For children with Spastic Quadriplegia Use motorized wheelchairs, special feeding devices, modified typewriters, and customized seating arrangements
Surgical To correct anatomical abnormalities or release tight muscles To help repair dislocated hips and scoliosis (curvature of the spine)
Surgery Dorsal rhizotomy (for severe spastic diplegia) – Cut specific nerves at their roots to reduce spasticity Stereotactic surgery – To improve rigidity, athetosis and tremors Reconstructive surgery to an arm – to restore muscle balance, release contractures, and stabilize joints
Medical Therapy Goal of pharmacotherapy is to reduce symptoms (e.g. spasticity) and prevent complications (e.g. contractures) 2 types of medications – For spasticity and abnormal movement – For seizures
Medications for spasticity Dopaminergic drugs – increase dopamine levels to decrease rigidity and abnormal movements – E.g. levodopa/carbidopa Botulinum toxin – Injected into specific muscle groups – Shows very positive response – May also be used to reduce the severity of drooling when injected into the salivary glands
Medications for spasticity Muscle relaxants – Baclofen: controls muscle contractions and relaxes tight muscles, but lowers seizure threshold – Botulinium toxin A: causes mild muscle paralysis and reduce contractions – Benzodiazepines (valium) – sedation is a side- effect – Oral dantrolene sodium – Need constant follow-up
Medications for seizures Anticonvulsants – Used to terminate clinical and electrical seizure activity as rapidly as possible – Prevent seizure recurrence Phenobarbital or phenytoin – Effective against partial seizures Benzodiazepines – Used in acute management of seizures
Other Problems Important to identify and manage behavioral problems early - work with a psychologist or psychiatrist Learning and attention deficit disorders, and mental retardation – assessed and managed by a psychologist and educator Strabismus, nystagmus, and optic atrophy are common – consult an ophthalmologist Promptly assess and treat lower urinary tract dysfunction Communication - Use Blissymbolics, talking typewriters, and specially adapted computers
Reference Nelson’s Textbook of Pediatrics 18 th ed. alsy