24 yr old mothers first child is now 2 yrs is sitting with support only. He scribbles with crayons. He crawls with abnormaly stiff leg movement.. she finds it difficult to change diapers. When held upright legs often cross so that she could not put ber on hips
She had regular antenatal check ups and scans. She was noted have high bp in 7th months. She had premature contractions . She received tocolytics. She delivered at 33weeks by lscs
Baby didnt cry soon aftet birth, resucitated and had seizures at 3 hours of life. Her cord ph was 7 bese deficit 12. Her apgar at 10minutes was 5at 10 minutes
Her stay in nicu lasted 7days with seizures, hypoglycemia and phototherapy
She was ahappy child doing things with hand and speech being normal.she could thread colored balls and stack color blocks
Development motor delay
Diet deficient in calories by 300 calories
Family socioe onomic status lower middle class
Ge exam nl
Head to foot
Motor spasticity of both lower limbs
Dynamic contracture +
Extensor plantar b/l
Equinus deformity +
Upper limbs normal tone
No meningeal signs
Cerebellar signs could not be tested
Assymetric tonic neck reflex present
Vision squint +, acuity?
Spastic diplegia, gmfcs 3/5,
Why this child is labelled as cp?
This child has antenatal risk factor, pih, prematurity followd by hie, neonatal seizures(post natal risk factor).The delayed motor development, persistent primitive reflex and lack of protective reflexes, spasticity and exaggerated dtr with associated squint has probably an insult to developing brain
How do you define cp?
Cerebral palsy (CP)1describes a group2of permanent3disorders4 of the development5 of movement and posture6 causing7 activity limitation,8 that are attributed to9 non-progressive10 disturbances11that occurred in the developing fetal or infant12 brain.13 The motor disorders of cerebral palsy are often accompaniedby14disturbances of sensation,15 perception16, cognition,17 communication, and behaviour, by epilepsy20, and by secondary musculoskeletal problems.21(rosenbaum)
What are causes of cp?
In 80% of cases, features were identified pointing to antenatal factors causing abnormal brain development.
Fewer than 10% of children with CP had evidence of intrapartum asphyxia.
What are the prenatal causes ?
Prenatal/ Neonatal Causes
• Prematurity a. Periventricular leukomalacia b. Intraventricular hemorrhage c. Bronchopulmonary dysplasia
• Hypoxic ischemic encephalopathy
• Neonatal hypoglycemia
• Congenital structural abnormalities
a. Inherited malformations b. Secondary to i. Infections ii. Radiation iii. Toxins like anti-epileptic drugs, alcohol, nicotine
• Multiple births
• Intracranial hemorrhage
• Intrauterine infections
• Neonatal infections
• Bilirubin induced neurological dysfunction
• Genetic susceptibility
What are the postnatal causes?
• Head trauma
• Hypoxic events like near drowning
• Febrile encephalopathy a. Sepsis b. Meningitis/ meningoencephalitis
• Status epilepticus sequelae
What are the early signs?
Conventionally many early signs are listed, but not many are objective
Prechtel general movement assessment and hine (hammersmith infant neurological exam ) are better predictors
Prechtel lack of fidgety movemnts and cramped assymetric movemwnts in 2-5months predicts cp
What are the conventional early signs?
Neurobehavioral signs: Neurobehavioral signs suspicious for CP are excessive irritability, lethargy, sleeps poorly, vomits frequently, is difficult to handle and cuddle, and has poor visual attention.
Developmental reflexes: Delay in disappearance or exaggeration of a developmental reflex may be an early indicator of motor disability
Motor tone and posture: Tone in extremities/ trunk may be normal, increased or decreased. Poor head control, persistent or asymmetric hand fisting, and abnormal oromotor patterns (tongue thrusting/ grimacing) may be the early motor signs.
Motor milestones: Serial examination of motor milestones is an effective screening process for picking CP early. T
Rasthriya Bal Swasthya Karyakaram (RBSK) of Government of India attempts to screen children from birth to 18 y of age for selected health conditions including defects at birth, deficiencies and developmental delays
In addition, a tool developed by INCLEN and improvised by AIIMS (AIIMS Modified INDT-NMI Tool) can be used for screening for neuromuscular impairments including cerebral palsy.
What are the types of cp?
What are the features spastic diplegia?
Spastic diplegia is bilateral spasticity of the legs that is greater than in the arms. Spastic diplegia is strongly associated with damage to the immature white matter during the vulnerable period of immature oligodendroglia between 20-34 wk of gestation.
The first clinical indication of spastic diplegia is often noted when an affected infant begins to crawl. The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal 4-limbed crawling movement. If the spasticity is severe, application of a diaper is difficult because of the excessive adduction of the hips. If there is paraspinal muscle involvement, the child may be unable to sit.
Examination of the child reveals spasticity in the legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign. When the child is suspended by the axillae, a scissoring posture of the lower extremities is maintained. Walking is significantly delayed, the feet are held in a position of equinovarus, and the child walks on tiptoe. Severe spastic diplegia is characterized by disuse atrophy and impaired growth of the lower extremities and by disproportionate growth with normal development of the upper torso.
The prognosis for normal intellectual development for these patients is good, and the likelihood of seizures is minimal. Such children often have learning disabilities and deficits in other abilities, such as vision, because of disruption of multiple white matter pathways that carry sensory as well as motor information.
The most common neuropathologic finding in children with spastic diplegia is periventricular leukomalacia (PVL), which is visualized on MRI in more than 70% of cases. MRI typically shows scarring and shrinkage in the periventricular white matter with compensatory enlargement of the cerebral ventricles.
What are the features of quadriplegua and hemiplegia?
Children with spastic quadriplegia have severe motor impairments. Both upper and lower limbs are affected nearly equally, and majority have very little speech and language development, visual impairment, epilepsy, and feeding difficulty. MRI in these children may show multicystic encephalomalacia.
In spastic hemiplegia, arm is typically affected more than leg. Athetotic posturing may accompany spasticity. Most children with spastic hemiplegia have associated sensory deficits. These sensory deficits are reflected as poor muscle mass on the affected side and do not correlate to motor deficits . In addition, they may have associated intellectual impairment, hemianopia, and other visual problems. Also, behavioral problems are common among children with hemiplegic CP including anxiety, oppositional defiance, and specific phobias.
What is the feature of dyskinetic cp?
Dyskineticsyndromes:PatientswithdyskineticCPoften have more than one form of involuntary movement. The limbs often become stiff during attempted movement or with emotion. Tendon reflexes may be normal or may be difficult to elicit. Athetoid movements of toes especially great toe (striatal toe) is a significant indicator of extrapyramidal dysfunction. Dyskinesia may also be seen in some spastic syndromes. However, unlike patients with spastic CP, children with purely dyskinetic syndromes do not develop contractures. The dyskinetic CP can be further sub-classified into:
Choreo-athetoid CP: It is characterized by rapid disorganized unpredictable contractions of individual muscles/ muscle groups involving face, bulbar muscles, proximal extremities and digits. In addition, they have slow writhing movements involving distal muscles. Oropharyngeal difficulties may result from facial grimacing. Primitive reflexes often persist into childhood. b) Dyskineticsyndromes:PatientswithdyskineticCPoften have more than one form of involuntary movement. The limbs often become stiff during attempted movement or with emotion. Tendon reflexes may be normal or may be difficult to elicit. Athetoid movements of toes especially great toe (striatal toe) is a significant indicator of extrapyramidal dysfunction. Dyskinesia may also be seen in some spastic syndromes. However, unlike patients with spastic CP, children with purely dyskinetic syndromes do not develop contractures.
The dyskinetic CP can be further sub-classified into: mass on the affected side and do not correlate to motor deficits . In addition, they may have associated intellectual impairment, hemianopia, and other visual problems. Also, behavioral problems are common among children with hemiplegic CP including anxiety, oppositional defiance, and specific phobias.
What are the d/d?
Despite advances in technology, cerebral palsy remains a clinical diagnosis, and represents a continuing role for the ‘‘art’’ of medicine.
The essential findings include:
Delayed motor milestones
Abnormal muscle tone
Absence of regression
A thorough history and physical examination should preclude a progressive disorder of the CNS, including degenerative diseases, metabolic disorders, spinal cord tumor, or muscular dystrophy. The possibility of anomalies at the base of the skull or other disorders affecting the cervical spinal cord needs to be considered in patients with little involvement of the arms or cranial nerves
What are the associated co morbidities?
Neurobehavioral/ neurodevelopmental disorders
impairment Growth failure Gastrointestinal disorders /Pulmonary disorders/ Orthopedic disorders/ Urinary disorders/ Pain /Sleep •
What are the investigations?
Should be done in all infants • Correlate MRI finding with history and clinical findings; if there is a discrepancy, rule out underlying metabolic disorde
An MRI scan of the brain is indicated to determine the location and extent of structural lesions or associated congenital malformations; an MRI scan of the spinal cord is indicated if there is any question about spinal cord pathology.
Should be performed in a child who is not gaining milestones (after an initial static insult) though the parents are denying any history of seizures
BERA and hearing assessment is a must for all infant
Visual assessment specifically for fundus assessment, strabismus with/without VEP should be performed
Genetic evaluation should be considered in patients with congenital malformations (chromosomes) or evidence of metabolic disorders (e.g., amino acids, organic acids, MR spectroscopy).
In addition to the genetic disorders mentioned earlier that can present as CP, the urea cycle disorder arginase deficiency is a rare cause of spastic diplegia and a deficiency of sulfite oxidase or molybdenum cofactor can present as CP caused by perinatal asphyxia
What is the treatment and prevention?
Some progress has been made in both prevention of CP before it occurs and treatment of children with the disorder. Preliminary results from controlled trials of magnesium sulfate, given intravenously to mothers in premature labor with birth imminent before 32 wk gestation, showed a significant reduction in the risk of CP at 2 yr of age.
Furthermore, several large trials have shown that cooling term infants with hypoxic-ischemic encephalopathy to 33.3°C for 3 days, starting within 6 hr of birth, reduces the risk of the dyskinetic or spastic quadriplegia
What is gmfcs? What is tardieu scale? What is macs? What is edac, vcfs?
What is tardieu scale for tone?
How do you manage motor impairment?
Physical therapy (PT) is an integral part of CP management and may range from a regular exercise program to variety of modalities including electrical stimulation.
Although its effectiveness in promoting physical function is uncertain, PT aids in encouraging caretakers to learn how best to handle, toilet, wash, and feed their children with CP and to promote posture,mobility, and transfer.
Occupational therapy (OT) techniques seek to improve function, but focus on maximizing a child’s ability to accomplish activities of daily living, education, and work. These are particularly helpful in maximizing available hand function
How do you manage spasticity?
Oral Medications Oral antispasticity agents including benzodiazepines, baclofen and tizanidine have the advantage of ease of administration but the disadvantages of systemic effect and significant sideeffects [10, 11]. Thus, they are most appropriate for children with generalized spasticity, and/or in children in whom only mild tone reduction is needed
What is thw role of
Inj botulinum toxin?
Botulinum toxin type A (BTX-A) when injected into the affected muscles, blocks the presynaptic release of acetylcholine from motor endplates of the lower motor neuron and decreases tone by limiting muscle contraction. Hence it produces chemodenervation.
The best evidence for use of BTX-A is for equines varus (treated by injections into the gastrocnemiussoleus muscles), but BTX-A is now used for a variety of focal indications including knee and hip flexion spasticity, and spasticity of the upper extremity. Patients younger than 4 y old and without fixed contractures are expected to have the most favorable response because the musculoskeletal and nervous system is most plastic in growing children
Phenol and Alcohol Phenol and alcohol are the other agents associated with chemodenervation, and have been used in children with CP,
Intrathecal baclofen (ITB), administered via acatheterattached to a programmable pump implanted subcutaneously, achieves higher cerebrospinal fluid drug levels as compared with oral administration, but may also be associated with substantial complication
What surgical procedures can benefit a child?
Selective Dorsal Rhizotomy (SDR) SDRisasurgical procedure thatselectively divides parts of the dorsal lumbosacral roots of the spinal cord. This interrupts the afferent limb of the reflex arc on the sensory side, thus reducing spasticity without causing paralysis. This technique may provide a small gain in function.
Deep Brain Stimulation
Deep brain stimulation (DBS) is primarily used for dystonias. Ameta-analysis of DBS in adults with dyskinetic CP suggests that it may be effective .
Despite best medical and rehabilitation management, children with spastic CP will often eventually require orthopedic surgery to correct deformities induced by muscle overactivity.
How to treat dystonia?
Dystonia and Mixed Movement Disorders Motor dysfunction in CP is not simply attributable to spasticity or corticospinal tract dysfunction; instead, it results from a disruption of the complex integration of multiple areas of the brain, including the cortex, cerebellum, basal ganglia, and brainstem. As a result, a child with CP may manifest other kinds of hypertonia and movement disorders.
Anticholinergic agent works at the level of the basal ganglia
Dopamine depletors, prevent neurotransmitter degradation
How to control drooling?
Oromotor dysfunction in children with CP often leads to drooling, which is a significant impediment to social acceptance . Approaches to improve drooling include pharmacotherapy, behavior-therapy, and surgery.
A trial of behavioral therapy is appropriate for children who are able to understand commands and cooperate with training. This consists of oral awareness and oral motor skills training, designed to improve lip and jaw closure and tongue movements with positive reinforcement for swallowing.
Pharmacotherapy for drooling includes anticholinergic drugs (trihexphenidyl or glycopyrrolate; act by decreasing flow of saliva), and BTX-A injection into salivary glands .
What are the assistive device useful forcp children?
Eye screeners for communication
Tablets with screen ipads
Hitech low tech communication boards
Apps for communication