CEREBRAL PALSY

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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% Seizures 25-35% Speech and language disorder 15-20% Extrapyramidal signs 10% Sensory impairment 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- Tetraplegia Diplegia Hemiplegia Monoplegia Neurological Dysfunction- Spastic Hypotonic Dystonic Athetotic or combination Site of brain lesion Periventricular Cerebellum 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 Neurological conditions: IVH ,PVL ,HIE Persistent neurological signs >1 week Global decreased density on CT scan APGAR Score 0-3 at 10,15and 20min Meningitis Persistent seizure Apnea beyond term Hyperbilirubinemia 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 Communication Education Nutrition Mobility 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. 3.Sensory Integration- -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 MEDICAL MANAGEMENT - Baclofen -Diazepam - 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 Cheap 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 Serial Casting- 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 Spasticity-dystonia Muscle imbalance GENERALISED SPASTICITY ITB(Intrathecal Baclofen) 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. Complications-Rare Infection Excessive bleeding Spinal cord injury Catheter migration 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 pure spasticity 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 *Hypotonic CP,Quadriplegia *Motor delay within 6 month *No sitting>4 yrs *Non ambulatory >7yrs *Persistent primitive reflexes >2yrs Good prognosis *Spastic diplegia *Sitting by 2yrs *Early intervention *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

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