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 %
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 mos…definite 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
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)