Cerebral Palsy

Cerebral Palsy: The most common motor disability in childhood

This is a fact-first primer on Cerebral Palsy. It aims to inform, and sensitise caregivers, peers and all readers about what this condition looks like, means medically, and the developmental differences it comes with. TSC will keep adding sections to this post to aid a complete and compassionate understanding of people born with this condition.

Cerebral Palsy (CP) is not just spasticity, neither is it always accompanied by an intellectual disability, and it is definitely not a sentence for the affected persons to live a life of complete dependence — 60% walk independently and about 8% use a hand-held device [1].

But, it is the most common and permanent motor disability starting in childhood.

The overall pooled prevalence of cerebral palsy in India [2] per 1,000 children surveyed is 2.95. Globally, according to the Centers for Disease Control and Prevention (CDC), it presents itself in at least 1.5 to 4 out of every 1,000 children.

Before we learn more about what qualifies as cerebral palsy, its causes, risk factors, types, diagnosis and treatment, let us understand that the prognosis of this condition does not change, it can’t be cured, and that it does not progress or become more severe with age. But, as the brain matures, the presentation of cerebral palsy, aka symptoms, changes and that can differ from person to person.

Cerebral palsy can affect a person’s posture, balance and ability to move, communicate, eat, sleep and learn. Early diagnosis, physiotherapy and other interventions greatly improve overall well-being and life outcomes.

What is Cerebral Palsy?

Cerebral palsy is a group of motor developmental disorders that permanently affect muscle tone, body movement, posture, and coordination. In many cases, vision, hearing, speech and sensation are also affected.

Cerebral (brain) + palsy (weakness)

This disability is caused by atypical brain development or injury to the developing brain before or after birth in the early development years (0-5 years). Though the exact cause of CP remains unknown in majority of the cases, here are some of the most common known causes [4], and risk factors [3].

Causes 

  • Asphyxia, or a lack of oxygen to the brain during labour and delivery
  • Gene mutation
  • Maternal infections, such as rubella, herpes, toxoplasmosis, cytomegalovirus
  • Severe jaundice as an infant
  • Brain infections, such as encephalitis and meningitis as an infant
  • Brain bleed, or haemorrhage
  • Head injuries as a result of accident, fall, or child abuse

Risk factors

  • Premature birth, or low birth weight
  • Being a twin or triplet or one of the babies in multiple gestations. Risk increases if one of the siblings dies before birth or soon after
  • Infertility treatments as they are known to result in pre-term delivery
  • Maternal medical conditions, such as abnormal thyroid function, pre-existing IDD, high protein in urine, seizures
  • Maternal exposure to toxic chemicals
  • Hypoxia
  • Jaundice in infants

Most children with Cerebral Palsy have the condition when they are born, but the symptoms are not visible, or noticeable, until months or years later. Delay in attaining movement milestones is among the biggest signs of CP and it usually becomes apparent before a child reaches 3-5 years of age.

Symptoms

Signs of cerebral palsy can vary greatly depending on the type and its severity. But, one of the biggest giveaways is the delay in reaching developmental milestones, such as rolling over, sitting, maintaining posture, crawling, standing, walking. Delay in speech development is a close second.

Here’s a complete checklist of developmental delays you should look for in infancy, and the years thereafter.

Up to 6 Months

  • Inability to support own head or head lagging back when picked up from supine position
  • Variation in muscle tone; body feeling too stiff or floppy
  • Tendency to overextend back and neck as if pushing away from the caregiver when cradled
  • Stiff legs that criss-cross or scissor when picked up

After 6 Months

  • Not rolling over in either direction
  • Inability to bring hands together, difficulty bringing hands to mouth
  • Not using both hands to reach out
  • Keeping one hand tightly fisted

At 10-12 months

  • Lopsided crawling, or not crawling on all fours, aka favouring one side of the body. For instance, using one hand and leg while dragging the other set
  • Scooting around on buttocks or knees

Other development delays and symptoms include

  • Stiff muscles, or spasticity
  • Permanent tightening of muscles, or contracture, especially of joints
  • Exaggerated reflexes
  • Lack of muscle coordination, or ataxia
  • Tremors or involuntary movements
  • Limited mobility of limbs
  • Abnormal gait
  • Walking on toe tips
  • Excessive drooling and problems with swallowing
  • Delays in speech development, difficulty speaking clearly

Contact a paediatric physician if you notice any of these signs. It is important to remember that not all children who have these symptoms may necessarily have cerebral palsy. These symptoms can be mild or severe, with the latter increasing the chances of co-occurrence with a range of other health conditions. Early diagnosis of the type of cerebral palsy and treatment — therapy, medication, surgery — are very important to improve quality of life.

Co-occurring conditions

A number of associated developmental delays and health conditions accompany cerebral palsy, but all of them may not always present simultaneously. 

  • Pain resulting in sleep and behaviour disorders
  • Incontinence
  • Epilepsy
  • Seizures
  • Intellectual disabilities, with autism spectrum disorder being the most commonly observed
  • Hip displacement
  • Spinal deformity such as scoliosis (curvature), lordosis (saddle back) and kyphosis (humpback)
  • Osteoarthritis
  • Poor bone density, or osteopenia
  • Dental problems
  • Visual impairment, or blindness
  • Hearing impairment
  • Speech impairment or remaining non-verbal

Types and Classification

Classification of cerebral palsy depends on three things [5]:

  1. Type of brain damage,
  2. extent of damage, or severity
  3. movement issues or affected body parts

Types of cerebral palsy are diagnosed by the region of brain injury and movement problem with the severity defining ability to live independently.

 
I. TYPE OF BRAIN DAMAGE

There are five major types of cerebral palsy based on the region of brain that is injured.  

Spastic Cerebral Palsy

Spastic/hypertonic/pyramidal cerebral palsy is the most common type accounting for 77% of all cases. It results in high muscle tone (hypertonia) causing limb stiffness and exaggerated, jerky movements, i.e. spasticity.

This type of cerebral palsy is caused by damage to the brain’s

  • Motor cortex, which controls voluntary movement. It is found on both sides of the brain
  • Pyramidal tracts, which relay signals to the muscles. The tracts connect the motor cortex on both sides to each other. Damage to the left motor cortex will affect the right side of the body and vice versa.

Commonly affected areas include arms and legs resulting in walking impairments, awkward reflexes, stiffness in one or many parts of the body. Contractures, or permanent tightening muscles or joints are also seen.

The tongue, mouth, and pharynx can also be affected, affecting eating, breathing, swallowing and speech.

This type of CP is considered to be the most severe, with a high prevalence of co-occurring conditions.

Dyskinetic

Dyskinetic/Athetoid, or non-spastic, cerebral palsy accounts for 2-3% of all cases. It results in hypotonia (loosened muscles) and hypertonia (stiffened muscles). The muscle tone can change from too tight to too loose several times in the same day.

This type of cerebral palsy is caused by damage to the brain’s

  • Basal ganglia, which controls voluntary motor function and eye movement
  • Cerebellum, which governs balance and coordination. 

It affects the extrapyramidal tracts that are responsible for involuntary reflexes signalled by the basal ganglia and cerebellum, thereby resulting in unintentional movement (twitches).

Commonly affected areas include the face, torso, and limbs, resulting in feeding issues, floppy limbs, rigid body, and posture problems. Sometimes the face and tongue are.

 

Ataxic

Ataxic cerebral palsy accounts for ~2% of all cases and causes ataxia — abnormal, uncoordinated movements, and/or tremors, jerks — and hypotonia (weakened muscles).

This type of cerebral palsy is caused by damage to the brain’s

  • Cerebellum, which is responsible for balance, coordinating physical movement

It commonly affects walking (spreading feet to steady themselves), balance, coordination, voluntary movement and fine motor functions, such as grasping objects, reaching out and writing, making individuals appear clumsy. It also affects depth perception and leads to speech difficulties.

 

Hypotonic

Hypotonic or atonic cerebral palsy accounts for 2-3% of all cases, and is classified by low muscle tone that results in loss of strength and firmness making muscles floppy, or lacking stability.

This type of cerebral palsy is also caused by damage to the brain’s

  • Cerebellum, which is responsible for balance, coordinating physical movement

If your child has hypotonic cerebral palsy they may face several physical or neurological impairments making them miss developmental milestones such as crawling, standing, or walking. It makes joints and ligaments flexible, and affects the head’s stability.

 

Mixed

Mixed cerebral palsy is the second most common type of cerebral palsy accounting for 15% cases.

This type of cerebral palsy is caused by damage to

  • Multiple areas of the brain

In such cases, when the damage is not confined to one location, a child may show symptoms of two or more types of cerebral palsy. The most common type of mixed CP is spastic-dyskinetic.

II. SEVERITY
The severity of different types of cerebral palsy is classified according to the Gross Motor Function Classification System (GMFCS) in 5 levels. It was developed by the World Health Organization (WHO) and the Surveillance of Cerebral Palsy in Europe as a universal standard to ascertain the physical capabilities.

The system focuses on capacity to:

  • sit
  • move
  • function independently
  • use of adaptive technology

A severe form of cerebral palsy may require a person to use specialised equipment to get around, such as a motorised wheelchair, and may need lifelong care. Or, a person with mild CP may be able to lift a fully independent life as an adult, including community engagement. 

Five levels as per the GMFCS system defining severity.

Level 1: Persons can walk without limitations.

Level 2: Can walk long distances with assistive devices, such as leg and arm braces but can’t run or jump. Such persons could require wheelchairs outside the house.

Level 3: They can stand without any support and sit with little support; need handheld assistive devices, like walker or crutch while walking indoors, and need wheelchairs outside the house

Level 4: Persons with level 4 cerebral palsy need assistive devices to walk, and can move independently in a wheelchair. But, they need support while sitting down.

Level 5: They need support to sit, stand, and maintain their head and neck position. They may be able to use a motorised wheelchair.

The symptoms of one person with the same level of cerebral palsy as another will be different depending on the type, co-occurring conditions, and affected areas of the body.

III. MOVEMENT PROBLEMS BASED ON AFFECTED BODY AREAS

There are six basic categories of movement issues based on the number of limbs, body parts or areas that are affected by the brain injury.

Monoplegia is a type of movement problem that occurs when only one arm or leg is affected. It is also very rare.

Diplegia affects two limbs — most commonly the legs — and mild movement issues in the upper body.

Hemiplegia affects one entire side of the body with the arm being more affected than the led, with a rigid wrist or elbow being the most notable feature.

Triplegia affects free movement of three limbs, and mostly presents itself in both legs and an arm.

Quadriplegia affects all four limbs with the legs being more impacted than the arms. It also limits use of facial muscles.

Double hemiplegia affects all four limbs are affected, but one side of the body is more severely impacted than the other.

Diagnosis

There is no one single conclusive test to diagnose cerebral palsy, it is hard to diagnose and the signs can go unnoticed till the child reaches 3 to 5 years of age (moderate-severe cases can be diagnosed around 2 years of age). The symptoms can appear at different ages for different children, and it is not until a child shows any of the mentioned motor delays that medical professionals can begin testing.

That said, some blood tests can be conducted for children in the high risk category to rule out hereditary conditions that affect motor function. Caregivers of high-risk kids must look out for developmental delays and other symptoms as well.

A complete diagnosis requires taking the complete medical history, evaluating symptoms, and a detailed physical and neurological examination which can include a battery of other diagnostic tests like MRI, CT scan, cranial ultrasound, EEG.

Despite being cumbersome, a complete diagnosis is important to:

  1. Remove doubt and fear of not knowing
  2. Understand the child’s health status
  3. Start early intervention and treatment to ensure best possible life for children with Cerebral Palsy

If a paediatrician indicates possible cerebral palsy after an exam, they may refer caregivers to a neurologist and orthopaedic doctor.

Once CP has been confirmed, the GMFCS is used to diagnose the severity of the condition, make a prognosis and determine the likelihood of a child improving their motor skills.

Children should also be tested to check for co-occurring conditions, such ID, hearing, speech and vision impairment, for holistic treatment.

The head injuries of younger children, and those with low-grade CP, may sometimes heal fully (there’s no cure), or show great improvement with correct treatment and early diagnosis.

Treatment

Cerebral palsy cannot be cured, but an early and complete diagnosis can help the child and the caregiver cope effectively with symptoms, prevent complications, and make the most of the child’s existing abilities through treatments, including:

Use of assistive aids: walking aids, wheelchairs, body braces, eyeglasses, hearing aids.

Medications: Oral anticonvulsants and muscle relaxants, like diazepam, dantrolene, baclofen, tizanidine, injections of botulinum toxin type A (Botox) or baclofen.

Surgery: Orthopaedic to correct bone abnormalities, improve mobility, relieve pain. Selective rhizotomy, a type of neurosurgical procedure, may be done to reduce spasticity.

Therapy: physical, psychotherapy, speech, recreational, occupational.

Stem cell therapy is being explored as a potential treatment for cerebral palsy, but the research is still in early stages.

References

[1]https://www.cdc.gov/ncbddd/cp/data.html#references

[2]https://www.researchgate.net/publication/334443310_Prevalence_of_Cerebral_Palsy_in_Indian_Children_A_Systematic_Review_and_Meta-Analysis

[3] https://www.nichd.nih.gov/health/topics/cerebral-palsy/conditioninfo/risk-factors

[4] https://www.cerebralpalsy.org/about-cerebral-palsy/diagnosis/checklist

[5] https://www.healthline.com/health/cerebral-palsy#risk-factors

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