Cerebral palsy describes a group of permanent disorders regarding development of movement and posture due to non-progressive disturbances (no longer active at the time of diagnosis) that occurred during fetal or infant brain development. The term ‘cerebral palsy’ is not an etiologic diagnosis (cause of a condition) but a clinically descriptive term. The disorder causes activity limitation, the motor disorders of cerebral palsy being accompanied by perception, sensation and cognition disturbances, alongside faulty communication and behavior, epilepsy and secondary musculoskeletal problems.

 

Bell’s palsy is an acute lower-motor-neuron facial nerve paralysis (idiopathic facial paralysis, or IFP), ‘idiopathic’ meaning ‘of unknown origin’. It is a temporary condition. When Bell’s palsy occurs, the function of the facial nerve is disrupted due to a miscommunication between the brain’s messages to the facial muscles, causing facial weakness or paralysis.

 

Progressive supranuclear palsy is a rare brain disorder (neurodegenerative disease) causing serious problems with control of gait and balance, eye movement and cognitive impairment. The disorder causes damage to the brain’s nuclei, structures involved in coordinating eye movement and frontal lobe, responsible for coordinating cognition. It is also known as the Steele-Richardson-Olszewski syndrome.

Causes

Cerebral palsy

Disturbances that occurred during fetal or infant brain development:

Random mutations

Maternal infections

Fetal stroke (disruption of blood supply to the developing brain)

Lack of oxygen to the brain (asphyxia) related to difficult labor or delivery (rare cause)

Infant infections causing inflammation

Traumatic head injury

Preterm birth

Congenital disorders

 

Bell’s palsy

An exact cause of the damage occurring to the nerve controlling facial muscles is still unknown

The facial muscles become swollen, inflamed, or compressed resulting in muscle weakening or muscle paralysis

Potential causes:

Viral infection

Chronic middle ear infection

Lyme disease

Trauma: skull fracture or facial injury

 

Progressive supranuclear palsy

An exact cause has yet to be identified

Researchers have found that patients suffering from PSP have abnormal amounts of tau cells buildup, a protein characteristic of other neurodegenerative disease, such as Alzheimer’s disease

Tau proteins are highly soluble and are abundant in neurons

There is no clear indication that the disorder is genetically transmittable

Symptoms and signs

Cerebral palsy

It is quite difficult to precisely label the resulting disability within typical subtypes, since the types occasionally overlap, many physicians having difficulty in properly diagnosing disorders due to the inexistence of a commonly recognized classificatory system

However, the most commonly used classificatory systems understood and used by physicians are:

Classification based on severity level

Classification based on topographical distribution

Classification based on motor function

Classification based on gross motor function classification system

 

Classification based on severity level (broad generalizations, lacking in specific criteria, but is useful for a first level understanding of the impairment):

Mild cerebral palsy (CP):

The child can move with no assistance

Daily activities are not limited

Moderate CP:

The child will need assistance in the form of braces, medications and adaptive technology to complete daily activities

Severe CP:

The child will require a wheelchair and will have to overcome major obstacles in accomplishing daily activities

No CP:

The child exhibits CP symptomatology, but the impairment was acquired after completion of brain development and is therefore classified under the cause of the CP – traumatic brain injury or encephalopathy

 

Classification based on topographical distribution:

It refers to the localization of the affected parts

It offers a description of the place and the degree to which the child is affected

The method is useful in determining treatment protocol

It makes use of a combination of two terms: paresis (=‘weakened’) and plegia/plegic (=‘paralyzed’)

Monoplegia/monoparesis:

Only one limb is affected

This is also believed to potentially be a form of hemiplegia/hemiparesis (where one limb is significantly impaired)

Diplegia/diparesis:

The legs are usually more affected than the arms

It primarily affects the lower body

Hemiplegia/hemiparesis:

The arm and leg on one side of the body are affected

Paraplegia/paraparesis:

The lower half of the body, including both legs are affected

Triplegia/triparesis:

Three limbs are affected: either both arms and a leg, or both legs and an arm, or it could also refer to one upper and one lower extremity and the face

Double hemiplegia/double hemiparesis:

All four limbs are involved

But one side of the body is more affected than the other

Tetraplegia/tetraparesis:

All four limbs are involved

But three limbs are more affected than the fourth

Quadriplegia/quadriparesis:

All four limbs are affected

Pentaplegia/pentaparesis:

All four limbs are involved

Neck and head paralysis are usually accompanying disabilities

Eating and breathing complications are resultant

 

Classification based on motor function and muscle tone:

Two main groupings include spastic (involuntary twitches) and non-spastic categorizations

Each has multiple variations, while it is possible to experience a mixture of both types

Motor function terms describe CP’s effect on muscle tone and the functioning of muscles together

When muscle tone is impaired muscles do not work together (one is relaxed, while the other is contracted) and can even work in opposition

Terms used to describe muscle tone: hypertonia/hypertonic (increased muscle tone, often resulting in limb stiffness, associated with spastic CP) and hypotonia/hypotonic (decreased muscle tone, often resulting in loose limbs, associated with non-spastic CP)

Spastic CP:

Increased muscle tone

Non-spastic CP:

Decreased or fluctuating muscle tone

 

Two classifications according to motor function:

Pyramidal (spastic) and extrapyramidal (non-spastic)

Pyramidal or spastic CP:

Consists of two groups of nerve fibers responsible for voluntary movements

They descend from the cortex into the brain stem

These nerves communicate electrical messages from the brain to the nerves located in the spinal cord in order to execute movement, thus actively stimulating the event

Spasticity implies increased muscle tone:

Muscles are continually contracting

Limbs are stiff, rigid and resistant to either flexing or relaxing

Reflexes may be exaggerated

Movements tend to be twitchy and awkward

Often affected are: the arms and legs, tongue, mouth, pharynx

Other signs and symptoms: impaired speech, eating, breathing and swallowing

Spastic CP is hypertonic

The stress created by spasticity can result in associated conditions such as: hip dislocation, scoliosis, limb deformities

Spastic CP is often named in combination with a topographical method that describes which limbs are affected (spastic diplegia, spastic hemiparesis, spastic quadriplegia)

Extrapyramidal or non-spastic CP:

Decreased and/or fluctuating muscle tone

Involuntary movements

The movement can be slow or fast, repetitive, and sometimes rhythmic

Planned movements can exaggerate gestures (a condition called intention tremors)

The injury is outside the pyramidal tract in places such as the basal ganglia, thalamus, cerebellum

Due to the location of the injury mental impairment and seizures are less likely to occur

It also lowers the likelihood of joint and limb deformities

Speech may be affected due to physical impairment, not intellectual

Non-spastic CP is also further classified into:

Ataxia/ataxic CP: affects coordinated movements, balance and posture. Walking gait if often very wide and sometimes irregular. Control of eye movements and depth perception can also be impaired. Coordination of eye to hands movement and writing are difficult

Dyskinetic CP is further divided into

Athetoid: involuntary movements of arms, legs and hands

Dystonia/dystonic CP: trunk muscles resulting in a twisted, rigid posture

Non-spastic CP may also be classified in terms of specific movement dysfunction:

Athetosis: slow movements, often repetitive, sinuous and rhythmic

Chorea: irregular movements, not repetitive or rhythmic, but more shaky and twitchy

Choreoathetoid: combination of chorea and athetosis, movements are irregular, but twisting and curving

Dystonia: involuntary movements, abnormal posture

Ataxia: does not produce involuntary movements but is associated with impaired balance and coordination

Mixed: the most common form of mixed CP consists in some limbs being affected by spasticity and others by athetosis

 

Classification based on Gross Motor Function System Classification:

It corresponds to a five-level system that corresponds to the extent of ability and impairment limitation

The higher the number, the higher the degree of diversity

GMFCS Level I:

Walks without limitations

GMFCS Level II:

Walks with limitations

Difficulties walking long distances and maintaining balance

May require use of mobility devices when learning to walk and when outside of home traveling long distances

GMFCS Level III:

Walks with adaptive equipment assistance

Hand-held mobility assistance needed when walking indoors

Wheeled mobility when walking outdoors

GMFCS Level IV:

Self-ability is possible only assisted by powered mobility

GMFCS Level V:

Severe head and trunk control limitations

Extensive use of assistive technology and physical assistance

Transportation via wheelchair

 

Bell’s palsy

Rapid onset of mild weakness to total paralysis of one side of the face

Facial drooping and difficulty making facial expressions (i.e. closing eyes, smiling)

Drooling

Pain around the jaw or behind the ear on the affected side

Increased sensitivity to sound on the affected side

Headaches

Decrease in the ability to taste

Changes in the amount of tears and saliva produced

In rare cases, it can affect the nerves on both sides of the face

 

Progressive supranuclear palsy

The onset of PSP is usually unobservable, but it includes a subsequent prolonged period of the following characteristics:

Fatigue

Headaches

Dizziness

Depression

Joint pain

Major symptoms:

Loss of balance while walking

Difficulty in controlling direction of gaze

Blurred or double vision

Additional signs mimicking Parkinson’s disease:

Stiffness and awkward movement

Repeated falling

Speech and swallowing difficulties

Sensitivity to light

Sleep disturbances

Impulsive behavior

Difficulties with memorizing, reasoning, problem-solving and decision-making

Depression and anxiety

A surprised or frightened appearance of facial expressions due to rigidity of facial muscles

Advice

Cerebral palsy

Long term care provided by:

Pediatrician

Pediatric neurologist

Orthopedic surgeon

Physical therapist

Occupational therapist

Speech-language pathologist

Developmental therapist

Mental health specialist

Social worker

Special education teacher

Drug treatments may also be prescribed for:

Isolated spasticity (to one muscle group)

Generalized spasticity

Bell’s palsy

Most people recover fully with or without treatment

Commonly used medications:

Corticosteroids – powerful anti-inflammatory

Anti-viral drugs

Physical therapy to treat shrunken, shortened muscles due to severe contractures

Decompression surgery is no longer advised due to the risk of permanent hearing loss and additional facial nerve injury

Progressive supranuclear palsy

No cure has yet to be identified

Treatments aim to ameliorate symptomatology:

Parkinson’s disease medication, but effects are temporary

Botulinum toxin type A injections in the muscles around the eyes

Eyewear with bifocal or prism lenses

Speech and swallowing evaluations

Physical therapy and occupational therapy for improving balance

MISCONCEPTIONS
  • Cerebral palsy

    Children with CP have a limited future

    There are not enough resources available for families to cope

    Children with CP will never be able to walk

    Children with CP will never be able to get a job – supported and mainstream employment rates are higher than ever

    Children with CP will not be able to lead a normal life – their livelihoods will be as similar to everybody else’s

    Independence is limited – assistive technology is as advanced as ever

    Bell’s palsy

    Stroke is usually thought of causing Bell’s palsy

    Flu shots can cause Bell’s palsy

    Progressive supranuclear palsy

    PSP is commonly misdiagnosed as Parkinson’s disease

Statistics

Cerebral palsy

The prevalence of CP in the United States, as well as internationally, is 1.5 to 2.0 cases per 1000 births. This rate has remained constant for nearly 40 years despite the technological advances decreasing mortality in both term and preterm infants (Clark and Hankins 2003, Gibson et al 2003)

Spastic CP accounts for nearly 70% to 80% of all cases of CP, with cognitive impairments seen in approximately 30% (Taft 1995, Thorogood 2001).

Non-spastic ataxic CP comprises 15% of all CP, while non-spastic dyskinetic CP comprises 5% of all CP (Sanger et al 2003, Taft 1995, Thorogood 2001)

 

Bell’s palsy

In the U.S. the annual incidence rate is approximately 23 cases per 100,000 people, with very few cases being observed during summer time

Internationally, the highest incidence has been noticed in Japan

Bell’s palsy is thought to account for about 60-75% of cases of acute unilateral facial paralysis, with the right side being affected in a majority of 63% of cases

It can also be recurrent with a reported recurrence range of 4-14%

It commonly occurs in adults

The highest incidence is in persons aged 60 or older

 

Progressive supranuclear palsy

The incidence of PSP in Australia has been identified as 3-4 million cases per year, while in the U.S. the incidence rate has been identified as about 1.39 cases per 100,000 population

The mean age of onset is of about 63 years of age

The median interval between onset and diagnosis is of approx. 3 years

PSP has a slight male predominance in most studies

 

Did you know?

Bell’s palsy

The natural course of recovering from Bell’s palsy divides patients into three groups:

Group 1 – complete recovery of facial motor function without sequelae

Group 2 – incomplete recovery of facial motor function, but with no defects visible to the untrained eye

Group 3 – permanent neurologic sequelae that are aesthetically and clinically visible

 

Progressive supranuclear palsy

PSP has been shown to become fatal in approximately 6 years after onset, mainly due to infections and pulmonary complications