Cerebral Palsy : Cerebral Palsy CEREBRAL PALSY : CEREBRAL PALSY Umbrella term covering a group of non progressive, but often changing motor impairment syndromes secondary to lesion or anomalies of brain arising in the early stages of its development
Characteristic feature is change in muscle tone and posture, both at rest and with voluntary activity INCIDENCE : INCIDENCE Incidence 1.5 -2.5/ 1000 live births
Associated features –
Mental retardation 50-75 %
Ocular involvement 50-70%
Behavioral problem 30-50%
Speech and language disorder 15-20%
Extrapyramidal signs 10%
Feeding difficulty AGE OF ONSET : AGE OF ONSET The brain lesions of CP occur from the fetal or neonatal period to up to age 3 years
Although the lesion to the developing brain occurs prior to age 3 years, the diagnosis of CP may not be made till that time
some children have been diagnosed with CP at an early age, only to have the symptoms resolve later
Some authorities advocate not making a definitive diagnosis until age 5 years or later CLASSIFICATION : CLASSIFICATION Topographical-
Athetotic or combination
Site of brain lesion
Midbrain GROSS MOTOR FUNCTION CLASSIFICATIONADL : GROSS MOTOR FUNCTION CLASSIFICATIONADL To standardize gross motor function in the CP child
Level 1-Walks without restrictions: limitations in more advanced gross motor skills
Level 2-Walks without assistive devices: limitations walking outdoors and in the community
Level 3- Walks with assistive mobility devices: limitations walking outdoors and in the community
Level 4-Self-mobility with limitations: transported or use power mobility outdoors and in the community
Level 5-Self-mobility severely limited even with use of assistive devices PATHOPHYSIOLOGY : PATHOPHYSIOLOGY The clinical presentation of CP may result from an underlying structural abnormality of the brain;
early prenatal, perinatal, or postnatal injury due to vascular insufficiency;
toxins or infections
pathophysiologic risks of prematurity.
Evidence suggests that prenatal factors result in 70-80% of cases of CP.
In most cases, the exact cause is unknown but is most likely multifactorial (Jacobsson, 2004). : Major events in human brain development and their peak times of occurrence (Volpe, 1995) include the following:
Primary neurulation - Weeks 3-4 of gestation
Prosencephalic development - Months 2-3 of gestation
Neuronal proliferation - Months 3-4 of gestation
Neuronal migration - Months 3-5 of gestation
Organization - Month 5 of gestation to years postnatal
Myelination - Birth to years postnatal severity of periventricular & intraventricular hemorrhage : severity of periventricular & intraventricular hemorrhage classification system originally described by Papile et al in 1978
Grade I - This is subependymal and/or germinal matrix hemorrhage.
Grade II - This is subependymal hemorrhage with extension into the lateral ventricles without ventricular enlargement.
Grade III - This is subependymal hemorrhage with extension into the lateral ventricles with ventricular enlargement. : Grade IV - A germinal matrix hemorrhage that dissects and extends into the adjacent brain parenchyma, irrespective of the presence or absence of intraventricular hemorrhage,
is also referred to as an intraparenchymal hemorrhage when found elsewhere in the parenchyma.
Hemorrhage extending into the periventricular white matter in association with an ipsilateral germinal matrix hemorrhage/intraventricular hemorrhage is termed a periventricular hemorrhagic venous infarction. Cerebral Palsy Diagnosis : Cerebral Palsy Diagnosis SUSPECT
Lethargy, or lack of alertness, Irritability or fussiness
Abnormal, high-pitched cry, Trembling of the arms and legs
Poor feeding abilities secondary to problems sucking and swallowing
Low muscle tone
Abnormal posture, such as the child favoring one side of their body
Seizures, staring spells, eye fluttering, body twitching
Abnormal reflexes. : Developmental delay, which means that a child is slower than normal to develop movement skills such as rolling over and sitting up
Neuromotor dysfunction, or delay in the maturation of the nervous system
Motor disability, indicating a long term movement problem
Central nervous system dysfunction, which is a general term to indicate the brain's improper functioning
Fisting after 2 months. Abnormal signs : Abnormal signs Reduced head circumference or fall in growth
Delayed social smile or poor head control
Delayed appearance of postural reflexes
Persistent primitive reflexes and ATNR
Increased tone, scissoring or abnormal posture
Visual problem :roving eyes, no visual following ,squint
Lack of auditory response LAB STUDIES : LAB STUDIES The diagnosis generally based on the clinical picture
coagulation disorders if a cerebral infarction is seen.
screening for an underlying metabolic or genetic disorder
Lactate and pyruvate values: abnormality of energy metabolism (i.e., mitochondrial cytopathy).
Thyroid function studies related to abnormalities in muscle tone or deep tendon reflexes or to movement disorders.
↑ ammonia levels may indicate liver or urea cycle defect.
Chromosomal analysis, including karyotype analysis and specific DNA testing: to rule out a genetic syndrome, if dysmorphic features or abnormalities of various organ systems are present. : EEG is important in the diagnosis of seizure disorders but is not indicated if seizures are not suspected along with CP.
Electromyography and nerve conduction studies are helpful when a muscle or nerve disorder is suspected (eg, a hereditary motor or sensory neuropathy as a basis for equinus foot deformities and toe walking).
Serum quantitative amino acid and urine quantitative organic acid values: These studies may reveal inherited metabolic disorders. IMAGING STUDIES : IMAGING STUDIES Cranial ultrasonography
performed in the early neonatal period can be helpful in medically unstable infants until they are able to tolerate transport for more detailed neuroimaging.
can delineate clear-cut structural abnormalities and show evidence of hemorrhage or hypoxic-ischemic injury. : CT scanning of the brain helps identify congenital malformations, intracranial hemorrhage, and periventricular leukomalacia in infants more clearly than ultrasonography.
MRI of the brain is the diagnostic neuroimaging study of choice for older children because it defines cortical and white matter structures and abnormalities more clearly than any other method. It also allows for the determination of appropriate myelination for a given age FAQs : FAQs Should neuroimaging be routinely obtained in CP? Scan abnormalities may determine an etiology in many children but there were insufficient data to assess this further. Scan abnormalities may occasionally (i.e., 5 to 22%) identify treatable conditions and may suggest an increased risk for associated conditions such as mental retardation and epilepsy. Contd… : Contd… MRI is more likely to be abnormal in cases of CP associated with prematurity, showing abnormalities such as periventricular leukomalacia, compared to infants born at term. An etiology of CP can be determined in many patients based on the results of neuroimaging in combination with the clinical history. Should metabolic or genetic testing be routinely ordered in children with CP? : Should metabolic or genetic testing be routinely ordered in children with CP? Metabolic or genetic causes for CP occur infrequently (i.e., 0 to 4%).
Metabolic and genetic studies should not be routinely obtained
atypical features in the history or clinical examination, metabolic and genetic testing should be considered
Detection of a brain malformation in a child with CP warrants consideration of an underlying genetic or metabolic etiology Epilepsy. Should EEG be routinely performed in the assessment of children with CP : Epilepsy. Should EEG be routinely performed in the assessment of children with CP Given the higher frequency of epilepsy in children with CP, EEG is often considered during the initial evaluation
No role of EEG in determining the etiology of CP nor in predicting the development of seizures in a child with CP.
No role predicting the development of seizures in a child with CP. American Academy of Neurology evidence classification scheme for determining the yield of established diagnostic and screening tests : American Academy of Neurology evidence classification scheme for determining the yield of established diagnostic and screening tests Neuroimaging is recommended in the evaluation of a child with CP if the etiology has not been established, for example by perinatal imaging .
MRI, when available, is preferred to CT scanning because of the higher yield of suggesting an etiology and timing of insult leading to CP. MANAGEMENT : MANAGEMENT Interventions for CP should be directed at maximizing the quality of life by improvement in daily function and reduction of the extent of disability. Initially, the parents and other caregivers should learn how to seat, dress, feed, communicate with, transfer, transport, and toilet the child. With growth and development, the child should achieve maximal independence in these activities. HIGH RISK INFANTS : HIGH RISK INFANTS Very low birth weight
IVH ,PVL ,HIE
Persistent neurological signs >1 week
Global decreased density on CT scan
APGAR Score 0-3 at 10,15and 20min
Apnea beyond term
Septicemia Multidisciplinary Approach : Multidisciplinary Approach multidisciplinary team to address the multiple medical, social, psychological, educational, and therapeutic needs.
Team members should have a goal-oriented approach
understanding and appreciation of contributions from all the disciplines.
Management should be directed at promoting the child's
Social and emotional development
Maximal independence in activities of daily living
Appearance as nearly normal as possible Occupational/Physical Therapy : Occupational/Physical Therapy Physical therapy is aimed at improving infant-caregiver interaction, giving family support, supplying resources, and parental education, as well as at promoting motor and developmental skills
Occupational Therapy (OT) focuses on the development of skills necessary for the performance of activities of daily living
OT also addresses cognitive and perceptual disabilities
Neurodevelopmental Therapy and Sensory Integration are specialized techniques used by the therapists to achieve these goals FAMILY COUNSELLING : FAMILY COUNSELLING Doctor should emphasize that CP is
* Not mental illness
* Not necessarily associated with MR
* Not contagious
* Not inherited
* Not curable
*Child with mild CP do well in main stream school but child with moderate to severe CP need to be educated in special school.
*Equal opportunities ,protection of rights and full participation
*Non discrimination common principles in many therapies : common principles in many therapies include development of sequence learning, normalization of tone, training of normal movement patterns, inhibition of abnormal patterns, and prevention of deformity.
In general, since a child learns motor control in a cephalocaudal direction, the therapist will work in the same way, first trying to establish trunk control and then working toward control of the lower extremities
However, the latest policy statement from the American Academy of Cerebral Palsy and Developmental Medicine concludes that the long-term benefits of Neurodevelopmental Therapy are marginal and not measurable Except for an immediate improvement in the range of motion of joints, no other functional parameters showed any long-term improvement.
A child with mild CP shows improvement with therapy, whereas the more severe cases progressively develop contractures and lever arm disease around the age of 4-7 years, after which, no further improvement occurs with continuing therapy. Therapy Approaches : Therapy Approaches Bobaths Neurodevelopmental Approach-
- Normalises tone,inhibit abnormal primitive reflex patterns.
-Facilitates automatic reactions and subsequent normal movements.
-Recommended for infants
-Emphasis on family involvement
-For older children emphasis to ADL skills. : 2.Roods sensorimotor approach-
-Goals to activate movement and postural responses at an automatic level while following developmental sequence.
-Specific sensory stimulation to elicit movement like icing,brushing.
-Introduced by Ayres
-Enhance development of pre-school and school age children : Enhances child’s ability to organise and integrate controlled sensory information such as-vestibular,proprioceptive and tactile stimulation
- Dantrolene BLOCKS : BLOCKS Botulinum Toxin type A
Injected into muscle
Lasts for 12-30 weeks
Easy to administer
In dynamic contractures causing functional impairment
No fixed contractures that would prevent functional impairment from occuring with botox induced tone induced tone reduction. PHENOL BLOCK : PHENOL BLOCK Injected into motor points of affected muscle
Lasts 4-12 months
Needs skill to administer
Can lead to haematoma formation
Painful ORTHOSIS : ORTHOSIS TRAFO(Tone Reducing AFO)
GRAFO(Ground Reacting AFO)-in crouch gait
Long leg braces as night splints
To reduce spasticity UNDERWATER TREADMILL : UNDERWATER TREADMILL Advantages of walking on a treadmill underwater instead of on land are that there is resistance from the water that improves leg strength, but the water is also supportive, so there's less joint stress. Warm water is also comfortable on the muscles. Hippotherapy (Horseback Riding Therapy) : Hippotherapy (Horseback Riding Therapy) Hippotherapy has been shown to improve gross motor function (tone, ROM, strength, coordination, and balance) which may reduce the degree of motor disability.
Hippotherapy offers many potential cognitive, physical, and emotional benefits, especially in post-surgical rehabilitation ASSISTED DEVICES : ASSISTED DEVICES Prone or supine standers , walkers ,bathing equipment , wheel chair
Augmentative communication computer
FUNCTIONAL ELECTRICAL STIMULUS
* Use of low voltage high frequency stimulus increases blood flow to muscles and improves muscles growth and strength. SURGICAL MANAGEMENT : SURGICAL MANAGEMENT SEMLS(Single Event Multilevel Surgery)
Indications(For Lower Limb)-
6-12 years of age
Any fixed deformity or contracture
Progressive hip subluxation
Functional mobility plateaus or regresses
When blocks less effective CONTRADICTION : CONTRADICTION Not before 6 years of age-Not necessary and will have to be replaced.
In teenagers-poor compliance and difficult rehabilitation.
In dystonia,ataxia and severe weakness.
In severely cognitive-behavioral impaired children
Poor family support
Poor physiotherapy support. Indications(Upper Limb) : Indications(Upper Limb) Contracture
Joint contracture and subluxation
For better function and cosmesis
8-12 years of age
Before flexion deformities become severe
For hygiene and care. Contraindications : Contraindications Asteroegnosis
SDR(Sacral Dorsal Rhizotomy) Intrathecal Baclofen Pump : Intrathecal Baclofen Pump Medical device to deliver very small quantities of medications directly to spinal fluid.
Medications like baclofen,mophine may be delivered to minimize side effects often associated with higher dosages in oral medications of same type
Potential candidates are-
- people with spastic diplegia or other forms of spasticity : Requires a surgical procedure.
Trial intrathecal injection or temporary pump to determine if the medication works.
Permanent is implanted by surgery if patient derives 50% improvement.
Spinal cord injury
Death during surgery(extrmely rare) Selective dorsal rhizotomy : Selective dorsal rhizotomy This neurosurgical operation involves a laminoplasty from L1 to S1 and section of 20% to 40% of the dorsal nerve rootlets
SDR only procedure that provides permanent reduction of spasticity in CP.
Neurosurgeon divides each dorsal roots into 3-5 rootlets and stimulates each rootlets electrically.
By examining electromyographic(EMG) responses from muscles in lower extremities, the surgical team identifies the rootlets that cause spasticity : The selection criteria includes
good selective motor control
adequate underlying muscle strength
age 4 years to 7 years
diagnosis of spastic diplegia due to prematurity
Abnormal rootlets are selectively cut,leaving the normal rootlets intact.
This reduces the messages from the muscle,resulting in a better balance of activities of nerve cells in the spinal cord,and thus reduces spasticity. RISKS : RISKS Paralysis of legs and bladder, impotence, and sensory loss are the most serious complications.
Wound infections and meningitis-controlled with antibiotics
Leakage of the spinal fluid through the wound. PROGNOSIS AND OUTCOME : PROGNOSIS AND OUTCOME Poor prognosis
*Motor delay within 6 month
*No sitting>4 yrs
*Non ambulatory >7yrs
*Persistent primitive reflexes >2yrs
*Sitting by 2yrs
*Good family support
*Absence of associated problems Future Prospect : Future Prospect Stem cell Transplantation
stem cell transplantation, that is aimed directly augmenting reparative abilities of an injured brain, opens new opportunities in the cerebral palsy treatment. By experimental and clinical investigations it is firmly established that when grafted into the injured brain, stem cells are able to ameliorate greatly injury-caused, neurological defects. SUCCESSFUL REHABILITATION : SUCCESSFUL REHABILITATION Prevention of additional impairments
Reduction in disability
Improved integration of child in community