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Ataxic Cerebral Palsy

Ataxic Cerebral Palsy

 

Cerebral palsy is the umbrella term for brain damage. The neurological disorders that can result are many and varied.

  • Nonspastic (extrapyramidal) cerebral palsy includes dyskinetic cerebral palsy (subdivided into athetoid and dystonic forms) and ataxic cerebral palsy.
  • Ataxic cerebral palsy is the rarest type of cerebral palsy and involves the entire body.
  • Ataxic cerebral palsy describes a condition indicating weak muscle tone.
  • Dyskinetic cerebral palsy (also includes athoid, choreoathetoid and kystonic cerebral palsies). This type of cerebral palsy involves fluctuating muscle tone.  Abnormal body movements affect the trunk, hands, arms and legs.

 

Causes

Ataxic cerebral palsy may be caused by a birth injury or birthing trauma, environmental factors, or by genetic, muscle, or central nervous system disorders such as down syndrome, muscular dystrophy, cerebral palsy, Prader-Willi syndrome, myotonic dystrophy or Tay-Sachs disease. Some children may experience hypotonia. This neuromuscular disorder is also referred to as “floppy infant syndrome” or “infantile hypotonia.” Individuals with this diagnosis are said to be “hypotonic” and demonstrate “hypo-tonicity.”

 

Cerebral Cortex

Imagine the cerebral cortex as the brain’s switchboard. Since the cerebral cortex contains nearly 75% of all the neuron cell bodies in the nervous system, injury to this portion of the brain would result in a variety of neuromuscular disorders. The effects of various injuries to the cerebral cortex depend on the location and severity of the damage. Doctors often describe the type of cerebral palsy a child has based on which limbs are affected.

 

Functions of the Cerebral Cortex

  • Cells in the upper portions of the cerebral cortex motor areas send impulses to muscles in the legs and thighs.
  • Cells in the middle portions control muscles in the shoulders and arms.
  • In the lower portions of the cortex, motor areas activate muscles of the head, face and tongue. Therefore, any injury to the motor system or pathways may result in a loss of the ability to produce purposeful muscular movements. The resulting disability would be classified as a “neuromuscular” disorder.
  • The primary motor areas of the cerebral cortex lie in the frontal lobes of the brain, therefore frontal lobe damage can result in ataxic cerebral palsy as well as a large variety of neurological disorders. An injury to the motor system may result in loss of ability of purposeful muscular movements (movement disorders) and can impair use of the hands, arms, legs, head or eyes.
  • The frontal lobes are also responsible for behavior and emotional development, therefore trauma to this portion of the brain may impair perception and rationality, behavior, personality, language skills, attention span, motor skills, facial expressions and sexual behavior.

The names of the most common forms of cerebral palsy use terms to describe the location or number of affected limbs, combined with the words for weakened (paresis) or paralyzed (plegia).

  • Hemiparesis (hemi = half) indicates that only one side of the body is weakened.
  • Quadriplegia (quad = four) means all four limbs are paralyzed.

The effects of ataxic cerebral palsy can range from complete loss of a function to compromised and diminished function. It is possible to determine the location of a cerebral injury by determining what functions and abilities are absent or diminished.

In the field of research, scientists are looking at traumatic events in newborn babies’ brains, such as bleeding, epileptic seizures, and breathing and circulation problems, which can cause the abnormal release of chemicals that trigger the kind of damage that causes ataxic cerebral palsy.

 

Midbrain

Cerebral insult or injury to the parasympathetic or sympathetic portions of the midbrain can result in ataxic cerebral palsy since the midbrain plays a role in facilitating motor function and muscle tone.

 

Cerebellum

Damage to the cerebellum portion of the brain can result in ataxic cerebral palsy and hypotonia, since the cerebellum facilitates the controlling and coordinating of muscles and helps with sustaining posture.

 

Diagnosis

Many physicians are reluctant to make a diagnosis of cerebral palsy until a child is 18 to 24 months old. Many of the normal developmental milestones are based on motor functions:

  • Reaching for toys (3-4 months)
  • Sitting (6-7 months)
  • Walking (10-14 months).

Some of the most pronounced movement disorders associated with ataxic cerebral palsy may not be evident until a child is expected to achieve certain developmental milestones. Parents are often the first to suspect that their baby’s motor skills are not developing normally and should contact their physician with any concerns since they can help distinguish normal variation in development from a developmental disorder. Most children with cerebral palsy are diagnosed by 3 years of age. Early assessment and intervention is vital in helping a child with this disorder meet developmental milestones.

 

Symptoms of Ataxic Cerebral Palsy

Imagine trying to walk, talk, swallow or perform any number of physical or autonomic functions with reduced or absent muscle tone.

  • This rare type of cerebral palsy affects balance and depth perception. Children with ataxic cerebal palsy will often have poor balance and coordination and experience challenges with precise movements. For example, the person may reach too far or too close to touch objects and may also have poor hand control (intention tremor).
  • A child with this form of cerebral palsy may walk with the feet unusually far apart and experience hand control issues, whereby only one hand is able to reach for an object; and the other hand may shake with attempts to move it (intention tremor).
  • A child may not be able to write or use scissors and may experience difficulty with quick or precise movements, such as writing or buttoning a shirt.
  • A child may display difficulties with sucking, swallowing, oral motor skills, breathing, delayed gastric and intestinal motility, further putting a child at risk of reflux and GERD
  • Children may also experience poor gross motor control, as well as impaired balance and depth perception.
  • It can also present issues involving a child’s ability to walk, sit, maintain torso control or use upper and lower extremities.

The hypotonic state of muscle tone that may be accompanied with ataxic cerebral palsy is not the same as muscle weakness, although the two conditions can co-exist. Depending on the nature, severity and location of the neurological damage, hypotonia can affect the entire body or be limited to parts of the body. It has the potential to impact cardiac function and bladder control and may influence pulmonary function by inhibiting diaphragm and respiratory muscle tone.

Children with this disorder may or may not exhibit development disabilities or mental retardation. In moderate to severe cases, a child may be at increased risk of contractures, spinal scoliosis and other muscular skeletal disorders.

 

Movement Disorder Symptoms

Movement Disorders associated with ataxic cerebral palsy may include tremors or jerky uncoordinated movements, which can occur when trying to exhibit control over affected muscles.

Children with ataxia cerebral palsy, or ataxic CP, have a disturbed sense of balance and depth perception, characterized by tremors or shaky movements. Depending on the nature and severity of the underlying cerebral injury, each case affects a child differently and some have more than one form of cerebral palsy (mixed cerebral palsy).

 

Prognosis

Ataxic cerebral palsy does not always cause profound disabilities. While one child with severe ataxic cerebral palsy might be profoundly delayed or unable to walk and need extensive, lifelong therapy and care, another with mild cerebral palsy might be only slightly inconvenienced and require no special assistance. Therapy, supportive treatments, medications and surgery can help many individuals improve their motor skills and ability to communicate with the world.

 

Treatment

Although there is no cure for the underlying neurological injury, children with ataxic cerebral palsy can benefit greatly from a variety of interventions:

  • Physical, occupational and speech therapy offers the venue to strengthen diminished or hypotonic muscle groups, and in some cases can help the brain establish new and different pathways to help facilitate a specific motor function.
  • Early intervention and management of the consequences of ataxic cerebral palsy is crucial in early childhood development. Left unaddressed, the absence of life skills which children rely on for so many of life’s demands can lead to severe developmental delays, a diminished quality of life and a severely compromised level of independence. Not to mention the behavioral issues a child will surely exhibit as a result of frustrations due to their inabilities. Consult your doctor for use of these therapies with other neurological disorders.

 

Ask Your Physician

Ask your child’s pediatrician what options are available to help your child avoid the possible consequences and complications that can result from ataxic cerebral palsy. If your child is not enrolled in an early intervention program, it’s NEVER too early. Many programs accept children under age 3. Most programs require a diagnosis, so if your child is diagnosed with ataxic cerebral palsy, get it in writing and ask your child’s physician to put you in contact with local and state service providers to begin the early intervention services and therapies your child can benefit from. Early assessment and services to address developmental issues are vital in allowing a child to reach their full potential.

Ask your child’s physician if your child would benefit from physical, occupation, speech or vision therapies, and request he write an order for services that he deems “medically necessary and appropriate.”

If a brain scan or MRI wasn’t done with the diagnosis, ask your child’s physician if a brain scan or MRI would be helpful to possibly better identify the cerebral injury and the possible long term consequences; proactive is always better then reactive. Many long-term crippling consequences of ataxic cerebral palsy can be minimized with diligence and the proper medications and therapies.

If your child has been prescribed medications, ask what follow up is needed with the medications; for example, lab work to monitor medication levels.

 

Resources

Floppy Infant Syndrome - http://www.ncbi.nlm.nih.gov/

Cerebral Palsy: Hope Through Research - http://www.ninds.nih.gov/disorders/cerebral_palsy/detail_cerebral_palsy.htm#154463104

Muscle Weakness (Hypotonia) - http://www.childrenshospital.org/az/Site1106/mainpageS1106P0.html

 

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2 Responses to “Ataxic Cerebral Palsy”

  1. valerie says:

    This will be so helpful because my 6y/o nephew has ataxic CP and his teacher does not believe us or his doctor and the school is contemplating expelling him because of behavioral problems and will not put him in a class for special needs kids and he has problems with frustration and lashes out i am printing this off and giving it to his teacher and maybe then she will understand that this is really a problem, and ataxic CP is a real thing

  2. Donna says:

    Good luck! and I just said same thing that I’m printing this and taking it to the school. Its a shame that the school system expects these children to be at school but then think they shouldn’t have a clue about CP. It sickens me! I have been having issues also and I am raising hell. I will make them aware and change because no child should be treated badly because they were born with a disability.