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| Division of Occupational Therapy - OT Connect Ataxic Cerebral Palsy & Cerebellar Ataxia
Cerebral Palsy (CP) is a disorder characterized by neurologic, motor, and postural deficits resulting from nonprogressive abnormalities in the developing brain. Ataxic CP occurs from brain damage to the cerebellum, and it affects sense of balance and depth perception. Individuals with this type of CP demonstrate low muscle tone and poor coordination of movements. This form is the rarest for of CP. It affects about 5-10 percent of the children diagnosed with cerebral palsy (About, 2004). Cerebellar Ataxia refers to a condition of unsteadiness of gate. Causes of ataxia are varied. It includes, among others, alcoholism, multiple sclerosis, brain tumors, thyroid disease and genetic abnormalities. It may also result from a viral infection or chicken pox during childhood. Various disorders fall under ataxia, including Freidrich’s Ataxia. Cerebellar Ataxia is caused by the degeneration of the cerebellum, the part of the brain that controls coordination and balance. Onset varies greatly from childhood to late adulthood. Childhood onset of Cerebellar Ataxia is more than likely caused by genetic abnormalities. Most of these cases are inherited in an autosomal recessive fashion, however, there have been rare cases where it has been traced to the X chromosome. Cerebellar ataxia, unlike cerebral palsy, may be progressive and the person may experience a loss of function over time. Common Symptoms/Course of disease Low muscle tone and poor coordination of movements characterizes ataxic cerebral palsy and cerebellar ataxia. Affected persons often have poor coordination and walk unsteadily with a wide based gait, placing their feet unusually far apart (About, 2004). Precise movements are also problematic, for example the person may reach too far or too close when trying to touch objects. They have tremors and shakiness, which make it difficult to complete fine motor tasks and manipulate small objects such as buttons, writing tools, and feeding utensils. Intention tremors lead to poor hand control because when the individual tries to move their hand it can cause shaking to occur (WebMD, 2003). Because of the shaky movements and problems coordinating their muscles, children with ataxic cerebral palsy may take longer than other children to complete certain tasks such self care tasks and handwriting (About, 2004). Age of Onset: Onset of ataxic cerebral palsy is before, during, or shortly after birth, or within the first two to three years of life (WebMD, 2003). Although the unusual movements and postures and other developmental problems may not appear until after the first year. These developmental problems do not mean that the condition is getting worse. Rather, they result from an existing problem that does not physically manifest until the nervous system matures (WebMD, 2004). Cerebellar ataxia occurs most often in adults, typically associated with another neurological condition such as: multiple sclerosis, brain tumors, thyroid disease and genetic abnormalities. It may also result from a viral infection or chicken pox during childhood. Sex Bias: None. Cultural Bias: None. Ataxic cerebral palsy is caused by an injury to the cerebellum and/or midbrain before, during, or shortly after birth. In many cases, no one knows for sure what causes the brain injury or what may have been done to prevent the injury. In general there are two problems that can cause cerebral palsy: failure of the brain to develop properly (developmental brain malformation) or neurological damage to the child's developing brain. Some causes of ataxic CP include infection (like meningitis), bleeding into the brain, and damage caused by lack of oxygen. The brain may fail to develop normally when it is deprived of blood, oxygen, or other nutrients. Premature birth and high or low birth weight are also linked to ataxic cerebral palsy (WebMD, 2003). Current Medical Treatment: "The treatment of cerebellar ataxia remains primarily a neurorehabilitation challenge, employing physical, occupational, speech, and swallowing therapy; adaptive equipment; driver safety training; and nutritional counseling. Modest additional gains are seen with the use of medications that can improve imbalance, incoordination, or dysarthria (amantadine, buspirone, acetazolamide); cerebellar tremor (clonazepam, propranolol); and cerebellar or central vestibular nystagmus (gabapentin, baclofen, clonazepam)." (Perlman, 2000) Occupational Therapy Involvement Occupational therapy works on control of the large and small muscle groups in the body. In this condition, occupational therapy can assist in developing control of any extraneous movements associated with the condition. Occupational therapists also assist with positioning needs to provide the physical stability needed to perform self-care skills and can adapt daily living techniques for tasks such as dressing and eating to meet the needs of the individual. Occupational Therapy may also be involved in teaching the child better or easier ways to write, draw, cut with scissors, brush their teeth, dress, and feed themselves (About, 2003). Active and passive ROM activities, positioning and handling to increase postural tone, and orthotics may be used to increase functional independence. Therapists can also help to educate parents on handling and positioning techniques for their child during routine activities such as sleeping, dressing, feeding, toileting, bathing, and carrying. The book, “Handling the Young Child with Cerebral Palsy at Home,” by Nancie R. Finnie describes in detail many handling techniques that can be utilized during these activities. Adaptive and assistive devices can be used to help children with ataxic CP to be more independent. The following are some low technology devices that may be used: Spoons or forks with heavy or large handles, spoons or forks that are curved or swivel, dishes with higher lips to help keep food from slipping, and cups with special handles or tops. If poor balance is keeping an individual from walking, a walker may be used to help them keep their balance so they can get around without the use of a wheelchair. Assistance in selection of toys that can be easily managed by the child and in leisure skills appropriate for the older individual are also within the scope of occupational therapy. In reviewing the limitations of the individual and the demands of their daily environments, the occupational therapist may also recommend higher technology devices to establish computer and telephone access, and access to powered mobility devices like scooters and wheelchairs (WebMD, 2003). In the older adolescent or adult, the occupational therapist can assist in driver training and automobile adaptations. References
How OT Makes a Difference: Evidence-Based Practice Blundell, S.W., Shepherd, R.B., Dean, C.M., Adams, R.D., & Cahill, B.M. (2003). Functional strength training in cerebral palsy: A pilot study of a group circuit training class for children aged 4-8. Clinical Rehabilitation, 17, 48-57. This study was done to determine the effects of intensive task-specific strength training on lower limb strength and functional performance in children with cerebral palsy. The subjects were 8 children aged 4-8 with spastic and ataxic cerebral palsy. Intervention consisted of an after-school exercise class and was provided one hour twice weekly for four weeks. Various workstations were set up for intensive repetitive practice of functionally based exercises including treadmill walking, step-ups, sit-to-stands, and leg presses. The results of the study showed an improvement in isotmetric strength from pre- to post-training by a mean of 47 percent and functional strength by 150 percent. The children walked faster and with longer strides, and showed on increase in sit to stand performance with a reduction of seat height from 27 to 17 cm. Eight weeks after the training, all improvements were still maintained. The conclusion of this study is that task-specific strengthening exercise and training improved strength and functional performance for children with cerebral palsy and this improved performance was maintained over time. This is a review article discussing progressive cerebellar ataxia in the adult population. It provides an excellent overview to the current understanding of the condition and recommends interventions for both primary and secondary impairments associated with the condition. From a mother with a child who has Ataxic Cerebral Palsy: “I have a 6 year old daughter with Cerebral Palsy. She was a premature baby and weighed very little when she was born. She weighed only 3 pounds and 1 ounce at the time she was born. When she was two years old she was diagnosed as having ataxic CP. Her muscles were very weak, and she did not start attempting to walk until she was about three and a half. She was always bumping into things and falling over. She now is able to walk by herself with the help of a walker to help with her balance problems and unsteadiness.” “She receives physical therapy and is working on walking without the use of a walker. She also receives occupational therapy, which is helping her to be able to use her hands better. The OT is working with her on self-care tasks such as buttoning and zipping clothing, brushing her teeth, and tying her shoes. Her therapist is also working on her handwriting and using objects such as pencils, scissors, and crayons. My daughter has problems with feeding also. Whenever she goes to move her food from the plate to her mouth, her hand starts shaking making it hard to feed herself. The OT also has been working with her on this, and we are trying some different types of utensils that may make it easier for her to use. I have seen many improvements since the OT and PT have been working with my daughter.”
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