Cerebral Palsy

CEREBRAL PALSY

Diagnostic term used to describe a group of motor syndromes resulting from disorders of early brain development.

Symptom complex, (not a disease) that has multiple etiologies.

Brain damageOccurs during developmental periodMotor dysfunctionNot CurableNon-progressive (static)

Any regression or deterioration of motor or intellectual skills should prompt a search for a degenerative disease

Therapy can help improve function

CP is caused by a broad group of

DevelopmentalGenetic Produce a common Metabolic group of neurologic phenotypesIschemicInfectiousOther acquired etiologies

CP is associated with

Epilepsy

Abnormalities speech, vision, intellect

Selective vulnerability of the brain’s motor system

Many children and adults function at a high educational level

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

• There is overlap of both symptoms and anatomic lesions.

Types of brain damage– Bleeding– Brain malformation– Trauma to brain– Lack of oxygen– Infection– Toxins– Unknown

Etiology Antenatal factors causing abnormal brain development

Congenital anomalies

Intrapartum asphyxia

Intrauterine exposure to maternal infection

Multiple births

Lowbirth weight infants

Intracerebral hemorrhage

Periventricular leukomalacia

Hypoxic Ischemic Encephalopathy (HIE)

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

encephalopathy.• Asphyxia refers to the first minutes after birth

(low Apgars and acidosis)• HIE signs and symptoms persist over hours

and days that follow.

Hypoxic Ischemic Encephalopathy (HIE)

3 major lesions arise from HIE1. Periventricular Leukomalacia (PVL) Typically seen in the

premature infant

a. Hemorrhagic PVL b. Ischemic PVL

2. Parasaggital Cerebral Injury Typically seen in the term infant

3. Selective (Focal) Neuronal Necrosis Seen in both term and premature infants

Periventricular Leukomalacia (PVL)

Hemorrhagic PVL

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

Periventricular Leukomalacia (PVL) Ischemic PVL• An ischemic infarction or failure of perfusion

usually to the watershed area surrounding the ventricular horns- “HIE white matter necrosis”.

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

cysts- “periventricular hemorrhage”.

Periventricular leukomalacia

Periventricular Leukomalacia (PVL)

Ischemic PVL• PVL can extend into the internal capsule and

result in hemiplegia superimposed on diplegia. • Prenatal maternal ultrasound has detected

lesions in the fetus at 28-32 weeks gestation, thus confirming that PVL can occur prenatally.

Parasaggital Cerebral Injury• Injury is related to vascular factors, especially in the

parasaggital border zones that are more vulnerable to a drop in perfusion pressure and immature autoregulation.

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

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

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

Selective (Focal) Neuronal Necrosis (SNN)

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

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

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

Types of Cerebral PalsyPyramidal• Velocity dependent increased resistance to passive muscle

stretch• The spasticity can be worse when the person is anxious or ill.• The spasticity does not go away when the person is asleep.

Extrapyramidal• Ataxia• Hypotonia• Dystonia• Rigidity

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

– During sleep the person is actually hypotonic

Types of Cerebral Palsy

Pyramidal (Spastic)• Quadriplegia- all 4 extremities• Hemiplegia- one side of the body• Diplegia- legs worse than arms• Paraplegia- legs only• Monoplegia- one extremity

Extrapyramidal

Divided into Dyskinetic and Ataxic types

Dyskinetic• Athetosis• Chorea- quick, jerky movements• Choreoathetosis- mixed• Hypotonia- floppy, low muscle tone, little movement

Ataxic CP• Results from damage to the cerebellum

• Ataxia- tremor & drunken- like gait

Anatomy

Pyramidal• Lesion is usually in the motor cortex, internal

capsule and/or cortical spinal tracts.

Extrapyramidal• Lesion is usually in the basal ganglia,

Thalamus, Subthalamic nucleus and/or cerebellum.

SPASTIC DIPLEGIA

Periventrıcular leukomalacia (PVL)PrematurityIschemiaInfectionEndocrine /Metabolic

SPASTIC QUADRIPLEGIA

PVL

Multicystic encephalamalacia

Malformation

infection

endocrine / metabolic

genetic / developmental

HEMIPLEGIA

Stroke inutero or neonatal

Thrombophylic disorders

Infection

Genetic/ developmental

Periventricular hemorrhage - infection

EXTRAPYRAMIDAL (athetoid-dyskinetic)

Pathology,putamen,globus pallidus,thalamus, basal ganglia

Asphyxia

Kernicterus Mitochondrial

Genetic/metabolic

SYMPTOMS

All types of CP are characterized by

Abnormal muscle tone

Reflexes

Motor development

Coordination

Classical SymptomsSpasticities

Spasms

Involuntary movements

Unsteady gait

Problems with balance

Scissor walking

Toe walking

Babies born with severe CP often have an irregular posture floopy or stiff

spinal curvature

small jawbone

SPASTIC HEMIPLEGA

Decreased spontaneous movements on the affected side

The arm is often more involved than the leg

Difficulty in hand manipulation is obvious by 1 yr of age

Walking is delayed until 18-24 months

Circumductive gait is apparent

Examination of the extreminites may show growth arrest

Spasticity is apparent in the affected extremities

An affected child often walks on tiptoe

Ankle clonus and a Babinski sign may be present

DTR are increased

1/3 of patients have a seizure disorder

25% have cognitive abnormalities

CT or MRI →An atrophic cerebral hemisphere with a dilated ventricule contrlateral to the side of the affected extremities

CT →Useful for detecting calcifications associated with congenital infections

Family histories suggestive of thrombosis and inherited clotting disorders may be present

SPASTIC DIPLEGIA

The most common form of the spastic forms

Bilateral spasticity of the legs

First indication is often noted when an infant begins to crawl = commando crawl

If the spasticity is severe application of diaper is difficult

Ankle clonus, Babinski sign ( bilateral)

Scissoring posture of the lower extremities

Walking is delayed

Child walks on tiptoe

Impaired growth of lower extremities

Hip problems,dislocations, strabismus

Normal intellectual development

SPASTIC QUADRIPLEGIA → ( TETRAPLEGIA)

Most severe form of CP

Motor impairment of all extremities

High association with mental retardation and seizures

Swallowing difficulties are common → aspiration pneumonia

Increased tone and spasticity

Brisk reflexes, plantar extensor responses

Speech and visual abnormalities

ATHETOID CP= EXTRAPYRAMIDAL CP

Less common

Affected infants are characteristically hypotonic with poor head control

Developed increased variable tone with rigidity and dystonia over several years

Feeding may be difficult

Speech is typically affected

Oropharyngenal muscles are involved

Seizures are uncommon

Can also be caused by kernicterus

DIAGNOSISHistory

Physical examinaton

Neurological examination

MRI → determine the location and extent of structural lesions,associated congenital anomalies

Hearıng and visual function test

Genetic evaluation

TREATMENT

Multidisciplinary approach in the treatment

Physians from various specialities

Occupational and physical therapist

Speech pathologist

Social workers

Educators

Developmental psychologist

Parents should be taught now to work with their children in daily activities Feeding

Carrying

Dressing

Bathing

Playing

Need to be instructed in the supervision of a series of exercises to prevent the development of the contractures

Spastic diplegia → treated initially with the assistance of adaptive

equipment such as walkers some surgical procedures that reduce

muscle spasm.

Quadriplegıa Motorized wheelchairs

Special feeding devices

Modified typewriters

Customized seating arrangements

Hemiplegia

Improved hand or arm functioning on the affected side

Orthopedic Problems

• Scoliosis• Hip Dislocations• Contractures• Osteoporosis

Medical Management

Oromotor Dysfunction• Especially common in persons with

Extrapyramidal CP and Spastic quadriplegia– Language delay/Speech delays– Drooling– Dysphagia– Aspiration

Medical Management

Gastrointestinal Dysmotility• Delayed gastric emptying• Gastroesophageal reflux– Pain– Chronic aspiration

• Constipation.

Medical Management

Gastrointestinal Dysmotility• Delayed gastric emptying• Gastroesophageal reflux– Pain– Chronic aspiration

• ConstipationThese disorders are interrelated and compound

one another.

Medical ManagementSpasticity Management

Management of spasticity does not fix the underlying pathology of CP, but it may decreased the sequelae of increased tone.

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

• scoliosis • hip dislocation • contractures

– Pain– Hygiene problems

Treatment of Spasticity

Medications• Valium• Dantrium• Baclofen• Clonidine• Clonazepam• BOTOX

Associated Problems• Mental Retardation• Communication Disorders• Neurobehavioral• Seizures• Vision Disorders• Hearing loss• Somatosensation (skin sensation, body awareness)

• Temperature instability• Nutrition• Drooling• Dentition problems• Neurogenic bladder• Neurogenic bowel• Gastroesophageal reflux• Dysphagia• Autonomic dysfunction