Cerebral Palsy

Dr.Reza Parker (MD Romania)

Definition• Cerebral palsy is a heterogeneous disorder of

movement and posture that has a wide variety ofpresentations, ranging from mild motorm disturbance to severe total body involvement

Etiology / Risk Factors

Cerebral palsy may result from an underlying structural abnormality of the brain; early prenatal, perinatal, or postnatal injury.

Prenatal

• Long menstrual cycle• Previous pregnancy loss• Previous loss of newborn• Maternal mental retardation• Maternal thyroid disorder, especially iodine deficiency• Maternal seizure disorder• History of delivering a child weighing less than 2000 g• History of delivering a child with a motor deficit, mental

retardation, or a sensory deficit

Perinatal

• Prematurity• Chorioamnionitis• Nonvertex and face presentation of the fetus• Birth asphyxia

Post Natal

• Infections (eg, meningitis, encephalitis)• Intracranial hemorrhage (eg, due to prematurity,

vascular malformations, or trauma)• Periventricular leukomalacia (in premature infants)• Hypoxia-ischemia (eg, from meconium aspiration)• Persistent fetal circulation or persistent pulmonary

hypertension of the newborn• Kernicterus

Risk Factors• preterm 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

There are three distinctive features common to all patients with cerebral palsy: • some degree of motor impairment, versus autism;• an impairment to the developing brain, making it

different from conditions that affect the mature brain in older children and adults; and

• a neurological deficit that is nonprogressive, versus other motor diseases of childhood, such as the muscular dystrophies

Classification

• Because of the wide variability in presentation and types of cerebral palsy, there is no universally accepted classification scheme

• Cerebral palsy can be classified by the:• Clinical physiological picture, the region of the body

affected, or• The neuroanatomical region of the brain that was

injured• temporally in relation to the time of birth, as

previously described

Geographical Classification

• The anatomical region of the body affected with the movement disorder should be identified

• It is difficult to completely classify because some extremities may be only subtly involved and a patient ’s pattern of involvement can change over time

Types

• Monoplegia• Hemiplegia• Paraplegia• Diplegia• Quadriplegia• Double Hemiplegia• Total Body

Monoplegia

• Monoplegia is very rare and usually occurs after meningitis

• Most patients diagnosed with monoplegia actually have hemiplegia with one extremity only very mildly affected.

Hemiplegia

• In hemiplegia, one side of the body is involved, with the upper extremity usually more affected than the lower extremity

• Patients with hemiplegia, approximately 30% of patients with cerebral palsy, typically have sensory changes in the affected extremities as well

Hemiplegia

• Severe sensory changes, especially in the upper extremity, are a predictor of poor functional outcome after reconstructive surgery

• Hemiplegic patients also may have a leg-length discrepancy, with shortening on the affected side, which can be treated with contralateral epiphysiodesis or leg lengthening

Diplegia

• Diplegia is the most common anatomical type of cerebral palsy, constituting approximately 50% of all cases

• Patients with diplegia have motor abnormalities in all four extremities, with the lower extremities more affected than the upper

Diplegia

• The close proximity of the lower extremity tracts to the ventricles most likely explains the more frequent involvement of the lower extremities with periventricular lesions

• This type of cerebral palsy is most common in premature infants; intelligence usually is normal

• Most children with diplegia walk eventually, although walking is delayed usually until around age 4 years

Quadriplegia• All 4 extremities are equally involved and many

patients have significant cognitive deficiencies that make care more difficult

• Head and neck control usually are present, which helps with communication, education, and seating

• Treatment goals for patients with quadriplegia include

• (1)a straight spine and level pelvis,• (2)located mobile hips with 90 degrees of flexion

for sitting and 30 degrees of extension for pivoting,

• (3)plantigrade feet that can fit in shoes,• (4)and an appropriate wheelchair1

Total Body

• Patients with total body involvement typically have profound cognitive deficits in addition to loss of head and neck control

• These patients usually require full-time assistance for activities of daily living and specialized seating systems to assist with head positioning

• Drooling, dysarthria, and dysphagia also are common and complicate care

Double Hemiplegia

• Some patients have a double hemiplegia pattern as a result of bleeding in both hemispheres of the brain

• It often is difficult to differentiate this from diplegia or quadriplegia; however, in double hemiplegia, the upper extremities typically are more involved than the lower.

Paraplegia

• Paraplegia is very rare and is characterized by bilateral lower extremity involvement with—in contrast to diplegia — completely normal gross and fine motor skills in the upper extremity

• Many patients diagnosed with paraplegia actually are diplegic with very mildly involved upper extremities

Physiologic Classification

• Most patients with cerebral palsy have recognizable patterns of movement that also can

• be classified • Thus an understanding of normal brain

developmenti s important • During the first trimester, the immature brain

separates into the gross structures, including the cerebrum, cerebellum, and medulla

Physiological Classification

• Neurons begin to form in the second trimester, and the total number of neurons an individual eventually has are present at the end of this time frame

• Any neurons lost from this point forward are• irreplaceable

• Synaptic connections and myelination begin during the third trimester and continue through adolescence in a highly organized fashion

• As these synapses develop, and myelinization continues, primitive reflexes disappear and more mature motor patterns arise

• Because of this continued development after birth, many injuries to the newborn nervous system go unrecognized until the absence of expected patterns can be detected

• Because different pathways of the brain are myelinated at different times,

• 1. spastic diplegia usually is not detected until 8 to 10 months of age;

• 2. hemiplegia, 20 months of age; • 3. and athetoid cerebral palsy, after 24 months of age

• It is important to keep this in mind because a child ’s pattern may change over time

Motor Syndromes • Spastic• Athetoid• Choreiform• Ataxic• Rigid• Hypotonic

Spastic

• Spastic is the most common form of cerebralpalsy, constituting approximately 80% of cases, and usually is associated with injury to the pyramidal tracts in the immature brain

• Spasticity, or the velocity-dependent increase in muscle tone with passive stretch, is caused by an exaggeration of the normal muscle passivestretch reflex

Athetoid• Injury to the extrapyramidal tracts and is characterized by dyskinetic,

purposeless movements that may be exacerbated by environmental stimulation

• The clinical picture varies based on the level of excitement of the patient

• In pure athetoid cerebral palsy, joint contractures are uncommon; the results of soft tissue releases, in contrast to those seen in spastic cerebral palsy, are unpredictable, and the procedures have a high complication rate

• With the improvements in prevention of Rh incompatibility leading to kernicterus, the incidence of athetoid cerebral palsy is decreasing

• Dystonia, characterized by increased overall tone and distorted positioning in response to voluntary movements, or hypotonia also can occur with athetoid cerebral palsy

Choreiform

• Choreiform cerebral palsy is characterized by continual purposeless movements of the patient ’ s wrists, fingers, toes, and ankles

• This continuous movement can make bracing and sitting difficult

Ataxic

• Ataxic cerebral palsy is very rare and probably is the most often misdiagnosed type

• It is characterized by the disturbance of coordinated movement, most commonly walking, as a result of an injury to the developingcerebellum

Rigif• Patients with rigid cerebral palsy are the most

hypertonic of all cerebral palsy patients • This hypertonicity occurs in the absence of

hyperreflexia, spasticity, and clonus, which are common in spastic cerebral palsy

• These patients have a “ cogwheel ” or “ lead pipe” muscle stiffness that often requires surgical release

• When a surgical release is done, it is essential not to overweaken the muscle, which would cause th opposite deformity to occur

Hypotonic

• Hypotonic cerebral palsy is characterized by weakness in conjunction with low muscle tone and normal deep tendon reflexes

• Many children who ultimately develop spastic or ataxic cerebral palsy pass through a hypotonic stage lasting 1 or 2 years before the true nature of their brain injury becomes apparent

• Persistent hypotonia can lead to difficulties with sitting balance, head positioning, and communication

Diagnosis

• History • clinical examination, • MRI• CT• Coagulation- for infarction• EEG• EMG/NerveConduction

Clinical Pictures

• Bladder and bowel control problems, digestive problems

• (gastroesophageal reflux)

• Skeletal deformities, dental problems

• Mental retardation and learning disabilities in some

• Seizures/ epilepsy

• Hearing and visual problems

• Sensory integration problems

• Failure-to-thrive, Feeding problems

• Behavioral/emotional difficulties,

• Communication disorders

Physical Examination

• In-depth assessment • 6 Subtests include:

– Reflexes– Stationary– Locomotion– Object Manipulation– Grasping, – Visual-Motor

Integration.

– The subtests yield a gross motor quotient

– a fine motor quotient– a total motor

quotient.• Ages covered: from birth

through five years of age

Denver Test II• Developmental Screening Test• Cover 4 general functions:

– personal social (eg. smiling), – fine motor adaptive (eg. grasping & drawing)– language (eg. combining words)– gross motor (eg. walking)

Ages covered: from birth to 6 years

CRITERIAPosturing / Poor muscle control and strength Oropharyngeal problemsStrabismus/ SquintTone (hyper-, hypotonia)Evolutional maldevelopment Reflexes (e.g. increased deep tendon) *Abnormalities 4/6 strongly point to

CP

Posturing / Poor muscle control and strength

• Test hand strength by lifting the child off the ground while the child holds the nurses hands.

• Observe for presence of limb deformity, as decreased use of extremity leads to shortening.

Upon extension of extremities on vertical suspension of the infant,

If infant back bend backwards like and arch

may indicate CP is severe

Oropharyngeal problems

Speech, swallowing breathing,drooling,

feeding poorly

Strabismus

Squint

Tone

• Hypertonia - rigid, tense

• Hypotonia – floppy or flaccid

Evolutional maldevelopment

• Delays in motor skills – such as rolling over, sitting, crawling, and

walking• Size for age. • Persistence of primitive reflexes or

parachute reflex fail to develop

• Present at birth, normally disappears after 3 or 4 months (some say 6months)

Alternative Names: Startle response; Startle reflex; Embrace reflex

Placing Reflex

• When the dorsal (back) side of the hand or foot is placed on the edge of a surface, such as a table, the infant will lift the extremity and place it on the flat surface.

Landau Reflex

• When the infant is held in a horizontal prone position, the infant will lift head and extend the neck and trunk. When the neck is passively flexed, the entire body will flex. This reflex is present by 6 months and hypotonicity (low tone) indicates motor system deficits.

- No treatment to cure cerebral palsy. - Brain damage cannot be corrected. • Crucial for children with CP:

–Early Identification; –Multidisciplinary Care; and –Support

A. General management - Proper nutrition and personal care

B. Pharmacologic Botox, Intrathecal, Baclofen

- control muscle spasms and seizures, Glycopyrrolate -control drooling

Pamidronate -may help with osteoporosis.

Baclofen• Delivered directly to the spinal fluid

• Using a pump

• To avoid brain effects

C. Surgery-To loosen joints,

-Relieve muscle tightness, - Straightening of different twists or unusual

curvatures of leg muscles - Improve the ability to sit, stand, and walk.

D. Physical Aids

Orthosis, braces and splints - Keep limbs in correct alignment - Prevent deformities.

Positioning devices -Enable better posture

Walkers, special scooters, wheelchairs - make it easier to move about.

E. Special Education

- To meet the child's special needs - Improve learning.

- Vocational training can help prepare young adults for jobs

F. Rehabilitation Services- Speech and occupational therapies may improve the ability to speak, and perform activities of daily living and to do some suitable works to have their own income.

G. Family Services

- Professional support helps a patient and family cope with cerebral palsy.

- Counselors help parents learn how to modify behaviors.

- Caring for a child with cerebral palsy can be very stressful.

- Some families find support groups helpful..

H. Other Treatment

- Therapeutic electrical stimulation,- Acupuncture,- Hyperbaric therapy - Massage Therapy might help

Hydrocephalus

• Hydrocephalus can be defined broadly as a disturbance of cerebrospinal fluid (CSF) formation, flow, or absorption, leading to an increase in volume occupied by this fluid in the central nervous system (CNS).

Pathophysiology

• Normal CSF production is 0.20-0.35 mL/min• most CSF is produced by the choroid plexus• which is located within the ventricular

system, mainly the lateral and fourth ventricles.

• The capacity of the lateral and third ventricles in a healthy person is 20 mL.

• Total volume of CSF in an adult is 120 mL.

CSF Production

• Normal route of CSF from production to clearance is the following:

• From the choroid plexus, the CSF flows to the lateral ventricle, then to the interventricular foramen of Monro, the third ventricle, the cerebral aqueduct of Sylvius, the fourth ventricle, the 2 lateral foramina of Luschka and 1 medial foramen of Magendie, the subarachnoid space, the arachnoid granulations, the dural sinus, and finally into the venous drainage.

• ICP rises if production of CSF exceeds absorption. • This occurs if CSF is overproduced, resistance to CSF

flow is increased, or venous sinus pressure is increased. CSF production falls as ICP rises.

• This may result in elevation of the corpus callosum, stretching or perforation of the septum pellucidum, thinning of the cerebral mantle, or enlargement of the third ventricle downward into the pituitary fossa (which may cause pituitary dysfunction).

Etiology• Brainstem malformation causing stenosis of the aqueduct of

Sylvius: This is responsible for 10% of all cases of hydrocephalus in newborns.

• Dandy-Walker malformation: This affects 2-4% of newborns with hydrocephalus.

• Arnold-Chiari malformation type 1 and type 2• Agenesis of the foramen of Monro• Congenital toxoplasmosis• Bickers-Adams syndrome: This is an X-linked hydrocephalus

accounting for 7% of cases in males. It is characterized by stenosis of the aqueduct of Sylvius, severe mental retardation, and in 50% by an adduction-flexion deformity of the thumb.

• Mass lesions: Mass lesions account for 20% of all cases of hydrocephalus in children. These are usually tumors.

• Hemorrhage: Intraventricular hemorrhage can be related to prematurity, head injury, or rupture of a vascular malformation.

• Infections: Meningitis (especially bacterial) and, in some geographic areas , cysticercosis can cause hydrocephalus.

• Increased venous sinus pressure: This can be related to achondroplasia, some craniostenoses, or venous thrombosis.

• Iatrogenic: Hypervitaminosis A, by increasing secretion of CSF or by increasing permeability of the blood-brain barrier, can lead to hydrocephalus. As a caveat, hypervitaminosis A is a more common cause of idiopathic intracranial hypertension, a disorder with increased CSF pressure but small rather than large ventricles.

• Idiopathic

Clinical Picture• Slowing of mental capacity• Headaches (initially in the morning) that are more significant than in

infants because of skull rigidity• Neck pain suggesting tonsillar herniation• Vomiting, more significant in the morning• Blurred vision: This is a consequence of papilledema and later of optic

atrophy• Double vision: This is related to unilateral or bilateral sixth nerve palsy• Stunted growth and sexual maturation from third ventricle dilatation:

This can lead to obesity and to precocious puberty or delayed onset of puberty.

• Difficulty in walking secondary to spasticity: This affects the lower limbs preferentially because the periventricular pyramidal tract is stretched by the hydrocephalus.

• Drowsiness

• Head enlargement: Head circumference is at or above the 98th percentile for age.

• Dysjunction of sutures: This can be seen or palpated.• Dilated scalp veins: The scalp is thin and shiny with easily visible veins.• Tense fontanelle: The anterior fontanelle in infants who are held erect

and are not crying may be excessively tense.• Setting-sun sign: In infants, it is characteristic of increased intracranial

pressure (ICP). Ocular globes are deviated downward, the upper lids are retracted, and the white sclerae may be visible above the iris.

• Increased limb tone: Spasticity preferentially affects the lower limbs. The cause is stretching of the periventricular pyramidal tract fibers by hydrocephalus

Diagnosis

• History• Clinical Examination• CBC, Genetic testing, Biochemical profiles, LP• CT. MRI

Treatment

Medications:• Decreasing CSF secretion by the choroid

plexus - Acetazolamide and furosemide• Increasing CSF reabsorption - Isosorbide

(effectiveness is questionable)

• Surgical-Shunting, Choroid plexectomy,