CEREBRAL PALSY

Myrna S. Fojas, MD Perinatal Ass of the Phil 2004

Definition
(Mackeith/Polani

1958) A persisting qualitative motor disorder appearing b/f the age of 3yrs due to a non-progressive damage of the encephalon before the growth of the CNS (Rang,1990,1993) CP is the result of damage to the developing brain w/c results in a disorder of movement and posture that is permanent but not unchanging

Pathway to progressive musculoskeletal pathology in CP

BTX-A mgmt of Spasticity in the context of Ortho surg for children withCP:HKGraham:European Journal of Neurology: Nov 2001

CNS lesion Upper motor neuron syndromespasticity + weakness/reduced activityfailure of longitudinal growth in skeletal muscle fixed contracture bony torsionjoint instability joint dislocation or degeneration
*

Contracture
The presence of spasticity increases muscle stiffness and inhibits growth The reduced or absent voluntary activities in weight bearing reduces the stretch on muscle w/c is a stimulus to grow

contracture develops b/c in the race between bone growth and muscle growth, the muscle is always doomed to 2nd place(Boyd/Grahaam,1997)

Musculoskeletal complication
The

secondary musculoskeletal pathology is NOT static but definitely PROGRESSIVE

Incidence
2-2.5/1000

live birth(no Phil. Data) Significant medical and economic impact on the affected individuals and society

Common Associated Problems

(1) Seizure (Epilepsy) (2) Mental retardation (3) Visual and hearing impairment (4) Growth retardation (5) Abnormal sensation and perception (6) Speech impairment

Different manifestations of CP
Etiology

and time of the CNS lesion Primary CNS pathology within the brain Clinical topography(hemiplegia, diplegia,quadriplegia) Associated impairment Secondary musculoskeletal pathology

classification
I. Spastic II. Exytrapyramidal III. Hypotonic IV. Mixed form Incidence (US data) 70 % 11.1 % rare 21.1 %

Spastic CP

Risk Factor:Spastic Diplegia

PREMATURITY 44%

of diplegic are premies

weighing < 2500 g

89%

of diplegic has history of intrauterine or perinatal ASPHYXIA

Fullterm Diplegic
prepartum cause birth asphyxia contributing factors 41 % 31 % 24 %

MENTAL RETARDATION and Diplegia

45 % fullterm Diplegia IQ = < 70

15 % premature Diplegia IQ = <70 *more severe the motor deficit, the more the MR *Sz 27% of diplegic

Musculoskeletal complications of Diplegia

TIP-TOEING GAIT=Contracture of the Achilles tendon=spastic,shortening of the gastrocnemius/soleus muscles CROUCH GAIT=Spasticity,contracture of the hamstring muscles SCISSORING= Spasticity of the adductors

*take note of specific muscle involvement* *contractures usually presents by 2yr

Spastic hemiplegia
Unilateral

paresis - right more than left involvement of arms > legs 4-6 mos..suspicious 15-18 mosdefinite dx( presence of hand preference BEFORE 2 yrs is pathologic)

Associated problems w/ Hemiplegia

(1) sensory abnormalities neglect position sense stereognosis (2) one side blindness (hemianopsia) (3) epilepsy 52 % appears < 18 months (4) IQ - 50 % average IQ 18 % above 100

Spastic quadriplegia
All

extremities, even trunk upper> lower has very few voluntary movement decerebrate rigidity
*Pathology: Extensive cystic degeneration of the periventricular white matter

Risk factors:Spastic Quadriplegia

(1) Prolong 2nd stage of labor (2) Precipitate delivery (3) fetal distress

Associated problems
(1) Pseudobulbar palsy - difficulties in swallowing (2) Vasomotor change (perspiration, BP) (3) Severe mental retardation (4) optic atrophy - poor to zero vision (5) Epilepsy - 50 %

EXTRAPYRAMIDAL CP
Defective

regulation of muscle tone and coordination damage to extrapyramidal system

Cause of extrapyramidal CP
58 % perinatal asphyxia

34 %

low birth weight (placental infarction / toxemia)

Types of extrapyramidal CP
A. Hyperkinetic choreaform choreoathetoid movements B. Dystonic -abnormal postures with or without tremors and myoclonus

Hyperkinetic
*Patients at risk Premature with asphyxia hyperbilirubinemia( jaundice)
*Natural History: Hypotonia w/ hyperreflexia during infancy Chorea/athetosis presents by 2-3 yrs up to 8 yrs

Dystonic
SGA with asphyxia in perinatal stage and last trimester of pregnancy

Eventual outcome of Extrapyramidal CP

1/2 will walk prior to 4 years(average 4 years 5 months) B. Impaired skilled hand movements(dressing,feeding difficulties) C.Speech defects because of lips,tongue,palate,resp.musc incoordination D. Aspiration risks E. Strabismus in >30%, nystagmus in 30% F.Seizures in 25% G.Hearing loss in 50% F. Mental retardation: 65%IQ>20, 45%IQ>90 Dystonic has worse IQ
A.

Mixed CP
Very

common spasticity and extrapyramidal

Summary
The

musculoskeletal complications of CP can be PROGRESSIVE CP has different manifestations depending on the area of the brain injured In Spastic and Dystonic CP, specific affected muscles can be identified Contractures develop by 2 yr

TREATMENT

Physical therapy alone

Doesnot

treat SPASTICITY which is permanent and unrelenting in CP Doesnot treat contactures but can delay its onset Major modality to strengthen the weak muscle which is of major importance in therapy Major help in balance, gait training

Orthopedic surgery alone

Does not treat spasticity because the muscle is continually enervated unless selective rhizotomy is done (a very selective procedure) Does not strenghten the weak muscle The only treatment for contracture but this may recur if spasticity is not eliminated Post operatively: 1)there is an increase dependence on assistive devises 2) walking speed decline dramatically while the energy cost of walking is increased 3)there is a much greater requirement for intensive rehab postoperatively just to get back to baseline before making functional gains

New Treatments which addresses Spasticity and Dystonia

BOTULISM

TOXIN INJECTIONChemodenervation of specific muscles to relieve spasticity (blocks Ach attachment on the muscle receptors)
Expensive

and 4-6 months effectivity

Intrathecal

Baclofen-reserved for the severe quadreplegic

Multimodal treatment
Symptoms Spasticity

Weakness contractures

Mode of therapy Spasticity mgmt(botox or intrathecal baclofen) PT(muscle strengthening/orthoses) Ortho surgery

Gastroc-Soleus (tip toeing)

Gastrocnemius

recession + BTX to soleus Followed by 6 wks plaster cast below the knee Extensive Rehab for muscle strengthening and lengthening

Summary
Prevention---good

perinatal care!! If spasticity/dytonia is/are present, treat the main muscles involved early to prevent contractures and complication by local Botox injection followed by intensive PT with or without surgery and casting (Botox may be expensive but in the long run will pay for itself)

Thank you