Cerebral Palsy

It is a static encephalopathy defined as a non-progressive disorder of posture and movement resulting from a lesion/defect of the developing brain.

It may be associated with:

• Epilepsy
• Abnormalities of speech
• Vision problems
• Intellectual disabilities

The causes are commonly categorized as:

• Prenatal (before birth)
• Natal (during birth)
• Postnatal (after birth)

Prenatal Causes

• Radiation injury
• Placental insufficiency
• Intra-uterine infection
• Exposure to drugs

Natal Causes

• Birth Asphyxia
• Congenital anomalies
• Trauma
• Prematurity

Postnatal Causes

• Metabolic conditions
• Trauma or infection in the immediate postpartum period
• Toxin-induced encephalopathy

Various forms of classification have been proposed. Recently, it has been classified as:

1. Physiologic: This classification examines the physiological aspects of cerebral palsy, particularly focusing on the motor and neurological impairments. It considers factors such as muscle tone, reflexes, and motor function. This classification is valuable for understanding the underlying neurological dysfunctions that contribute to the condition.
2. Topographic: The topographic classification categorizes cerebral palsy based on the specific body regions affected by motor impairments. It helps pinpoint which anatomical areas are impacted, providing insights into the localization of motor difficulties.
3. Functional: Functional classification assesses the functional limitations and how they affect an individual's daily life. It takes into account how motor disabilities impact activities of daily living, mobility, and overall quality of life. This classification is crucial for designing tailored interventions and support.
4. Aetiologic: Aetiologic classification focuses on identifying the causes and contributing factors that lead to cerebral palsy. It encompasses prenatal, perinatal, and postnatal factors, including genetic, environmental, and medical influences. Understanding the etiology of cerebral palsy can guide preventive measures and targeted treatments.

Physiologic Classifications:

• Spastic: Characterized by muscle stiffness and difficulty moving.
• Atonic: Marked by low muscle tone, leading to poor coordination and posture.
• Rigid: Involves stiff and inflexible muscles, making movement challenging.
• Athetoid: Results in involuntary, writhing movements and difficulty holding posture.
• Tremor: Causes rhythmic shaking or tremors in the body.
• Ataxic: Affects balance and coordination, resulting in shaky movements.
• Mixed: Combines features of multiple classifications.
• Unclassified: Some cases may not fit neatly into specific categories.

Mnemonic: SARATAMU

Topographic Classifications:

• Monoplegia: Involves one limb, typically an arm or leg.
• Diplegia: Affects both limbs on one side of the body, often the legs.
• Triplegia: Involves three limbs, usually both legs and one arm or one leg and both arms.
• Quadriplegia: Affects all four limbs, including both arms and legs.
• Hemiplegia: Affects one side of the body, including both the arm and leg on that side.
• Double Hemiplegia: A more severe form of hemiplegia, affecting both sides of the body.
• Paraplegia: Primarily affects both legs, while the arms and upper body are not significantly involved.

Aetiologic Classifications:

• Pre-natal: Cerebral palsy is caused by factors that occur before birth, such as prenatal infections, genetic factors, or maternal health issues.
• Natal: Cerebral palsy is caused by factors related to the birth process itself, such as birth asphyxia or trauma during delivery.
• Post-natal: Cerebral palsy is caused by factors that occur after birth, such as infections, head injuries, or toxins that affect the developing brain.

Functional Classifications:

1. Class 1: Little or no limitation of muscular activities
2. Class 2: Mild to moderate limitation of muscular activities
3. Class 3: Moderate to severe limitation of muscular activities
4. Class 4: No useful muscular activities in the affected muscles.

GMFCS (Gross Motor Function Classification System) Levels:

1. Level I: Walks indoors & outdoors without limitation. Some difficulties in complex (running, jumping) gross motor activities. Difficulties with uneven surfaces.
2. Level II: Walks indoors without assistive mobility device. Climbs stairs holding railing. Unable to run or jump.
3. Level III: Walks with an assistive mobility device on level surfaces, climbs stairs with adult assistance. Frequently transported when traveling for long distances or outdoors.
4. Level IV: May walk short distances with a walker and supervision, with difficulty turning & maintaining balance on uneven surfaces. Transported in the community. May achieve self-mobility using a power wheelchair.
5. Level V: Physical impairments restrict voluntary movement. All areas of motor function limited. No means of independent mobility.

The Manual Ability Classification System (MACS)

The Manual Ability Classification System (MACS) is a classification system used to describe the manual abilities of individuals with cerebral palsy and similar conditions. MACS classifies individuals into one of five levels based on their self-initiated ability to handle objects in daily activities. Here's the classification system:

• Level I: Handles objects easily and successfully.
• Level II: Handles most objects but with somewhat reduced quality and/or speed of achievement.
• Level III: Handles objects with difficulty; needs help to prepare and/or modify activities.
• Level IV: Handles a limited selection of easily managed objects in adapted situations.
• Level V: Does not handle objects, and has severely limited ability to perform even simple actions.

• The symptoms vary significantly, but in most cases, they are essentially those of upper motor neuron lesions.
• Decreased spontaneous activities.
• A child less than 1 year will demonstrate hand preference.
• Delayed walking may be a feature.
• Spastic hemiplegia may have a circumductive gait.
• Growth arrest in extremities.
• Tip-toeing activity.
• Commando crawling is common in spastic diplegia.
• The mother may experience difficulty applying a diaper.
[insert videos]
• When suspended, there is a demonstration of scissoring posture.
• Spastic quadriplegia is the most severe form.
• It is often associated with mental retardation, hypereflexia, and hypertonia.
• Flexion contraction is seen at the knee in late childhood.
• Speech abnormality is common.
• In Athetoid CP; poor head control, feeding difficulty, tongue thrust, drooling of saliva, and slurred speech are features.
• There is impairment of voice modulation due to the involvement of oropharyngeal muscles.

Co-Morbid Conditions

• Learning vs Cognitive Disability
• Attention Deficit
• Oromotor Dyspraxia
  • Communication Impairment
  • Feeding Difficulty
• Visual Impairment

A thorough history and physical examination.

History

1. Birth and Perinatal History:

• Document gestational age, birth weight, and multiple births.
• Inquire about prenatal factors, including maternal infections, medication use, and exposure to toxins.
• Assess the circumstances of labor and delivery, including any complications, use of forceps or vacuum extraction, and signs of birth asphyxia.

2. Developmental Milestones:

• Gather information on early developmental milestones (e.g., sitting, crawling, walking) and identify any delays.
• Explore the onset and progression of motor difficulties, such as abnormal muscle tone or delayed motor skills.

3. Medical and Surgical History:

• Document any medical conditions or surgeries the child has undergone, especially those related to neurological or orthopedic issues.
• Note any seizure history, its onset, frequency, and treatment.

4. Family History:

• Investigate a family history of neurological disorders or genetic conditions.
• Explore the presence of consanguinity or hereditary patterns that may be relevant.

5. Social and Environmental Factors:

• Assess the child's living environment, including accessibility and safety concerns.
• Evaluate the family's support system, access to healthcare, and socioeconomic status, as these factors can impact care and rehabilitation.

6. Medication and Therapy History:

• Record current and past medications, particularly those aimed at managing spasticity, seizures, or associated conditions.
• Document any ongoing therapies, such as physical, occupational, or speech therapy.

7. Functionality and Quality of Life:

• Discuss the child's abilities and limitations in daily activities, mobility, and communication.
• Assess the impact of CP on the child's and family's quality of life and emotional well-being.

8. Social and Educational History:

• Explore the child's educational and social experiences, including school attendance, classroom support, and social integration.

9. Assistive Devices and Orthotics:

• Document the use of assistive devices, mobility aids, and orthotic interventions.
• Evaluate their effectiveness in improving function.

10. Parental Concerns and Expectations:

• Encourage parents or caregivers to express their concerns, expectations, and goals for their child's care and future.

• 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.
• Additional studies may include tests of hearing and visual function.
• Genetic evaluation should be considered in patients with congenital malformations (chromosomes) or evidence of metabolic disorders.

Physical Examination

1. General Assessment:

• Observe the child's general appearance, posture, and body habitus.
• Assess for signs of discomfort, pain, or distress.
• Note any involuntary movements or muscle spasms.

2. Neurological Examination:

• Evaluate muscle tone in all extremities, noting any hypertonia (spasticity), hypotonia (flaccidity), or dystonia.
• Assess for abnormal reflexes, such as hyperactive tendon reflexes (e.g., exaggerated knee jerk reflex).
• Test for clonus, an involuntary, rhythmic muscle contraction and relaxation, particularly in the ankles.
• Observe for the presence of primitive reflexes that typically disappear in infancy but may persist in CP.

3. Motor Function Assessment:

• Observe the child's range of motion in all joints, paying attention to contractures or joint deformities.
• Evaluate muscle strength and coordination.
• Assess the child's ability to perform gross and fine motor tasks.
• Document any asymmetric motor deficits.

4. Functional Mobility:

• Assess the child's mobility and ability to ambulate, including their gait pattern.
• Note any use of mobility aids, orthoses, or assistive devices.
• Evaluate balance and coordination during activities such as standing, walking, or reaching.

5. Communication and Speech:

• Evaluate the child's speech and language abilities.
• Assess for any difficulties in articulation, phonation, or comprehension.
• Determine the child's preferred communication mode, whether verbal, non-verbal, or augmentative and alternative communication (AAC) systems.

6. Sensory Examination:

• Test sensory perception, including touch, pain, temperature, and proprioception.
• Assess visual and auditory function, as sensory impairments are common in children with CP.

7. Orthopedic Evaluation:

• Examine for musculoskeletal abnormalities, including joint contractures, scoliosis, and hip displacement.
• Consider the need for imaging studies such as X-rays to assess bone and joint integrity.

8. Respiratory Assessment:

• Assess respiratory function, particularly in children with severe motor impairments who may be at risk of respiratory complications.
• Monitor for signs of respiratory distress or aspiration.

9. Swallowing and Feeding Evaluation:

• Evaluate the child's ability to swallow safely and efficiently.
• Assess nutritional status and the need for dietary modifications or feeding interventions.

10. Behavioral and Psychological Assessment:

• Consider the child's emotional and psychological well-being.
• Address any behavioral challenges or mental health concerns.

Diagnostic Imaging:

• 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.

Additional Studies:

• Additional studies may include tests of hearing and visual function.

Genetic Evaluation:

• Genetic evaluation should be considered in patients with congenital malformations (chromosomes) or evidence of metabolic disorders.

• Because CP is usually associated with a wide spectrum of developmental disorders, a multidisciplinary approach is most helpful in the assessment and treatment of such children.
• Treatment is multidisciplinary, coordinated by the Pediatrician. Other members of the team include Occupational Therapist, Speech Pathologist, Social Worker, Special Educator, Developmental Psychologists, Physical Therapist, and understanding and caring parents.

Drugs Useful in Cerebral Palsy

• For those with spasticity, useful drugs include benzodiazepines, dantrolene sodium, and Intrathecal Baclofen.
• For severe spasticity – botulinum toxin (BOTOX).
• Levodopa is useful in those with incapacitating athetosis.
• Carbamazepine is useful in Dystonia.

[insert video]

• It is a subset of developmental disabilities defined as significant delay in 2 or more of the following developmental domains:
• Gross/fine motor
• Speech/language
• Social/personal
• Activities of daily living
• Significant delay is defined as performance that is 2 standard deviations or more below the mean.
• The term global developmental delay is reserved for younger children (under 5 years) while the term mental retardation is usually applied to older children among whom IQ testing is more valid and more reliable.