I.  ATAXIC CEREBRAL PALSY (ATAXIA)

Ataxia is the least common form of cerebral palsy. Ataxia means ‘without order’ or ‘incoordination’.
Ataxic movements are characterized by clumsiness, imprecision, or instability. Movements are not smooth and may appear disorganized or jerky.
The incoordination seen with ataxia occurs when a person attempts to perform voluntary movements such as walking or picking up objects. Ataxia causes an interruption of muscle control in the arms and legs, resulting in a lack of balance and coordination.

        People with ataxia may have:

  • Unsteady, shaky movements or tremor
  • Difficulties maintaining balance
  • People with ataxia appear very unsteady and shaky because their sense of balance and depth perception is affected.


What does ataxia look like?

Ataxia can affect any part of the body and impact upon the movements required to do many day-to-day activities.
It can affect a person’s legs, arms, hands, fingers, speech, eye movements and even muscles involved in swallowing.
Effect on the upper limbs (arms and hands)

When ataxia affects the arms and hands it may cause a tremor or shakiness due to the over-correction of inaccurate movements – this means that when a person reaches for an object, they overshoot the target.

It also results in difficulty performing tasks requiring precise finger movements such as handwriting or using cutlery, or movements that require regular repetition such as clapping.

Effect on the lower limbs (legs)

When ataxia affects walking, a person is unstable and likely to fall. As a result, the person usually walks with the feet spread further apart than the hips, which is known as a ‘wide-base gait’. This is done to try to compensate for their instability and poor balance.

This way of walking can sometimes give the mistaken impression that the person is under the influence of alcohol or drugs. Because their balance is affected, the person may also fall without reason, or be unable to compensate for being accidentally bumped or for variations in the ground surfaces or an accidental mild bump from the side.

Effect on speech and swallowing

Ataxia may have an effect on speech and swallowing. When ataxia affects speech, it is sometimes called ‘scanning’ speech – the person uses a monotone voice with a breathy sound; sometimes there are unusual accelerations or pauses between their syllables.

Effect on the eyes

Ataxia may sometimes cause slow eye movements. When the person attempts to change their eye-gaze quickly, their eyes may miss the target. The eyes overshoot or underestimate their mark and then have to make ‘catch-up’ movements.




References:

Pakula, A. T., Van Naarden Braun, K., & Yeargin-Allsopp, M. (2009). Cerebral palsy: classification and epidemiology. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 425-452. doi: 10.1016/j.pmr.2009.06.001 See abstract
Paneth, N. (2008). Establishing the diagnosis of cerebral palsy. Clinical Obstetrics and Gynecology, 51(4), 742-748. doi: 10.1097/GRF.0b013e318187081a See abstract

(Update: 6 April 2016)

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II.    DYSKINETIC CEREBRAL PALSY (DYSKINESIA)

People with dyskinetic forms of cerebral palsy have variable movement that is involuntary (outside of their control). These involuntary movements are especially noticeable when a person attempts to move.

Dyskinetic movements can be:

Twisting and repetitive movements – known as dystonia
Slow, ‘stormy’ movements – known as athetosis
Dance-like irregular, unpredictable movements – known as chorea.

Dyskinetic movements often co-occur alongside spasticity.

What causes dyskinesia? Dyskinetic cerebral palsy results from damage to the basal ganglia of the brain. The basal ganglia is like the brain’s switchboard for interpreting messages between the movement centre and the spinal chord – it is responsible for regulating voluntary movements. The different forms of dyskinesia result from damage to slightly different structures within the basal ganglia.  [basal-ganglia-damage-large]

Dystonia

Dystonia is characterised by involuntary muscle contractions that result in slow twisting or repetitive movements, or abnormal sustained postures, that are triggered by attempts to move.

       Characteristics of dystonia include:

  • Repetitive and sustained movements
  • Awkward postures
  • Movements that are rapid or slow and are often painful
  • Involuntary movements triggered by attempts at controlled movement
  • Involuntary movements occur more frequently when the person is tired, anxious, tense or emotional
  • Pain may also result in an increase in these movements


What part of the body is affected?

Dystonia can be present in only one part of the body, known as focal dystonia, or throughout the whole body, known as generalized dystonia.
Focal dystonia may only occur during a particular movement or task. For example, dystonia in the foot muscles may affect how a person stands on that foot.
Cervical dystonia is another example of a focal dystonia – this is dystonia in the neck muscles which causes the neck to twist, tilt or rotate.
Hemidystonia is where on arm and one leg on the same side of the body are affected by dystonia.

Generalized dystonia refers to dystonic movement that affects both legs and at least one arm, or the trunk in combination with at least one arm or leg. Generalized dystonia can affect mobility as well as cause speech and swallowing difficulties.

Sensory tricks

Some people with dystonia notice that specific sensations and movements, known as ‘sensory tricks’, temporarily suppress their unwanted movements.

        Examples of sensory tricks include:

  • Touching the face or chin with a hand or finger
  • Resting the back of the head against a wall
  • Tucking the hand under the chin
  • Placing the hand behind the back


Some people use these sensory tricks to dampen an unwanted movement in order to prepare their body to make a desired movement.

Athetosis

Athetosis is characterised by slow, continuous, involuntary, writhing movements that are present at rest and made worse by attempts to move.

People with athetosis experience fluctuations in muscle tone – with muscle tone alternating between being floppy (hypotonia) and extremely variable motion (hyperkinesias).

One difficulty caused by this fluctuation in muscle tone is the inability to maintain a posture. This means that sitting and standing still can be difficult and a person may need to work and concentrate harder than usual to get their hand to a certain spot (like scratching their nose).

        Characteristic of Athetosis include:

  • Noticeable slow and stormy, involuntary muscle movements
  • Can cause a person to appear restless and constantly moving
  • Unwanted movements may be small or big, rapid, irregularly repetitive, random or jerky
  • Slow, writhing movements associated with athetosis usually affect the hands, feet, arms or legs
  • The involuntary and uncontrollable movement fluctuations sometimes affect the whole body
  • Muscles of the face and tongue can also be affected causing grimacing and drooling
  • Athetosis can affect a person’s ability to control lip and tongue movements, breathing and vocal cords. Speech can be affected to some degree in people with athetosis
  • Similarly, a person with athetosis may experience difficulties with eating and drinking
  • A person with athetosis is often only able to be still when they are fully relaxed
  • Athetoid movements ususally disappear completely when a person is asleep
  • Athetoid cerebral palsy makes it difficult to hold onto an object, like a pencil or cutlery, due to fluctuating muscle tone
  • The abnormal movement caused by athetosis often increases in response to emotional stress.


Chorea

The term chorea is derived from the Greek word for ‘dance’. Chorea is characterised by involuntary movements that are brief, abrupt, irregular and unpredictable. People with mild chorea may appear fidgety or clumsy whilst people with more severe choreiform movements may display wild, violent movements that are large in amplitude (ballismus).

Movements can affect various body parts and interfere with movement, speech and swallowing.

Chorea may worsen with attempts to move and anxiety or stress. Movements subside during sleep.

Choreiform movements may occur with athetosis and when this occurs, they are known as choreoathetosis. They may also occur with dystonia.




References:

Sanger, T. D., Chen, D., Fehlings, D. L., Hallett, M., Lang, A. E., Mink, J. W., Valero-Cuevas, F. (2010). Definition and classification of hyperkinetic movements in childhood. Movement Disorders, 25(11), 1538-1549. doi: 10.1002/mds.23088

(Update: 08 April 2016)

III.  SPASTIC CEREBRAL PALSY (most common)

Spastic cerebral palsy is the most common type of cerebral palsy. The muscles of people with spastic cerebral palsy feel stiff and their movements may look stiff and jerky.

Spasticity is a form of hypertonia, or increased muscle tone. This results in stiff muscles which can make movement difficult or even impossible.

Muscles appear stiff because the messages to the muscles are sent incorrectly through the damaged part of the brain
When a muscle is affected by spasticity, the faster the limb is moved, the stiffer it seems
Spasticity arises as a result of damage to bundles of neurons in the brain and spinal cord called the corticospinal tractsand corticobulbar tracts
Spasticity is seen in a number of different conditions including cerebral palsy, traumatic brain injury, spinal cord injury, stroke and multiple sclerosis
People may have difficulty moving from one position to another and controlling individual muscles or muscle groups needed for performing certain tasks like handling objects or speaking

What causes spasticity?  


In cerebral palsy, spasticity is due to damage to the motor cortex of the brain before, during or after birth. [motor-cortex-damage-large]

What does spasticity look like?

Spasticity may affect any muscle group in the body however there are some common patterns that are seen in cerebral palsy.

Effect on the upper limbs (arms and hands)

       If spasticity affects one or both of the arms it can lead to:

  • Flexion at the elbow (the elbow is bent)
  • Flexion at the wrist (the wrist is bent)
  • Flexion at the fingers (the fingers are fisted)


       Spasticity in these areas can lead to difficulties with tasks involving the hands and the arms:

  • getting dressed
  • washing and toileting oneself
  • eating or drinking
  • writing
  • manipulating objects


It may also affect a person’s ability to use their arms for balance which can lead to difficulty standing and walking.

Effect on the lower limbs (legs)

        If spasticity affects one or both of the legs it can lead to:

  • Flexion at the hip (which causes the leg to lift upwards when lying or the body to lean forwards in standing)
  • Adduction or ‘scissoring’ of the thighs (which causes the legs to pull together)
  • Flexion at the knees (causing changes in a person’s standing posture)
  • Equinovarus foot posture (where the toes point downwards and inwards with the heel off the ground – this results from tightness in the calf muscles)


       Spasticity in the muscles of one or both legs may affect a person’s ability to

  • Stand upright
  • Sit upright
  • Transfer from one position to another
  • Move and reposition in bed
  • Walk and run


Effect on speech

Spasticity can also be present in smaller muscles or muscle groups such as the tongue, facial muscles or vocal folds.

        Spasticity in these areas can result in:

  • Slow oral movements that are imprecise and require a lot of effort (eating and drinking may also be affected)
  • Speech sounds that are slurred
  • A voice that might sound hoarse or tight


This can impact a person’s ability to communicate using speech and may mean that alternative methods of communication (such as pictures, symbols or voice generating devices) are used.

What can change over time?

Although the damage to the brain that causes spasticity does not change over time, the effects of spasticity on the body can result in changes.

        Effects of spasticity over time:

  • Changes in soft tissues (muscles, tendons and ligaments) leading to muscle stiffness, atrophy (deterioration or wasting of the muscle) and fibrosis (changes in the properties of the muscle fibres)
  • Muscles that are affected by spasticity have difficulty stretching out to keep up with bone growth – resulting in muscles that are shorter than they should be. This prevents a joint achieving its normal full range of movement and is called a contracture.
  • Shortened, contracted muscles can pull on the bony structures of the body leading to bone deformities such as scoliosis of the spine and hip dislocation.
  • Pain – persistent overactivity in spastic muscles can cause pain in the muscle. Pain can also occur as a result of changes in the joint position and deformities due to the abnormal pull of the spastic muscle.


How is spasticity assessed?

An accurate and comprehensive assessment is important in determining the presence, severity and impact of spasticity. It is also important in evaluating an individual’s response to spasticity management intervention.
When assessing spasticity, a clinician will assess

Which muscles are affected by spasticity
Severity of the spasticity in each of the affected muscles
How a person is able to control their muscles (can they activate individual muscles, can they voluntarily control their muscles to perform functional tasks)
Any secondary effects of spasticity (such as contracture) which are affecting the person’s ability to perform functional tasks.

A clinician will also complete a clinical examination involving ‘hands on’ feeling for stiffness when moving a limb, measurement of range of movement of the joints, as well as strength, and
A functional assessment which will include observation of the person performing daily activities in a natural environment with, or without the use of equipment or other aids (eg bathing, dressing, sitting, walking, climbing stairs)




References:


Pakula, A. T., Van Naarden Braun, K., & Yeargin-Allsopp, M. (2009). Cerebral palsy: classification and epidemiology. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 425-452. doi: 10.1016/j.pmr.2009.06.001 See abstract


Paneth, N. (2008). Establishing the diagnosis of cerebral palsy. Clinical Obstetrics and Gynecology, 51(4), 742-748. doi: 10.1097/GRF.0b013e318187081a See abstract

(Update: 08 April 2016)