Cerebral Palsy CEREBRAL PALSY Diagnostic term used to

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 damage Occurs during developmental period Motor dysfunction Not Curable Non-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 Developmental Genetic Metabolic Ischemic Infectious Other acquired etiologies Produce a common group of neurologic phenotypes

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 HIE 1. 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 Palsy Pyramidal • 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 & drunkenlike 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) Prematurity Ischemia Infection Endocrine /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 Symptoms Spasticities 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

DIAGNOSIS History 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 • Constipation These disorders are interrelated and compound one another.

Medical Management Spasticity 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