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 2-2.5 cases per

1000 live births– In developing countries: about 1.5-5.6 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

• Sensory:– Sensorineural and conductive hearing loss– Impaired visual acuity– Oculomotor dysfunction– Strabismus– Cortical visual impairment– Somatosensory impairments

Associated Conditions

• Cognitive and linguistic:– Mental retardation– High incidence of language and learning

disabilities– Dysarthria– Attention deficit hyperactivity disorder– Sleep and behavioral disturbances

Associated Conditions

• Neurologic:– Seizures– Hydrocephalus

• Musculoskeletal:– Contractures– Hip dislocation– scoliosis

Associated Conditions

• Cardiorespiratory:– Upper airway obstruction– Aspiration pneumonitis

• GI/Nutritional:– Poor growth– Gastroesophageal reflux– Constipation– Dysphagia

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 SYNDROME NEUROPATHY MAJOR CAUSES

Spastic Diplegia PeriventricularLeukomalacia (periventricular

leukomlacic [PVL])

PrematurityIschemiaInfectionEndocrine/metabolic (e.g.,

thyroid)

Spastic Quadriplegia PVLMulticystic encephalomalaciaMalformations

IschemiaInfectionEndocrine/metabolicGenetic/developmental

Hemiplegia Stoke: in utero or neonatal Thrombophilic disordersInfectionGenetic/developmentalPeriventricular hemorrhagic

infearction

Extrapyramidal (athetoid, dyskenetic)

Basal gangliaPathology: putamen, globus

pallidus, thalamus

AsphyxiaKernicterusMitochondrialGenetic/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

Treatment

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 18th ed.• http://www.emedicinehealth.com/

cerebral_palsy• http://emedicine.medscape.com