CEREBRAL PALSY ATHETOSIS or Dystonic CP

General Features
Athetosis describes a child whose whole body passes into a state of slow, writhing muscular contractions when attempt any voluntary movement. When he is trying to use his hands, his head turns, his face grimaces, his mouth opens, both arms pass into a series of contraction, his trunk squirms, and in many cases he cannot control his leg movements. The abnormal movements in this from of cerebral palsy can be explained only in terms of an abnormal reflex pattern. The athetoid child may show undue persistence of the Moro reflex and the asymmetric tonic neck reflex. A sudden noise may send him into severe spasm, involving the whole body and if he is able to stand, he may lose his balance. All athetoid children, even the mildest case, will show remains of the asymmetric tonic neck reflex. When the head is turned to one side the jaw arm extends, the skull arm flexes and in many cases the legs are also involved. In most athetoid the normal balance reflexes are not present. If the child is pushed over, he cannot put out his hands or abduct his legs to save himself. The ease with which these children are knocked over may give an impression of ataxia, particularly if hypotonia is present. The tone of the muscles in Athetosis may vary from severe spasm to apparent hypotonia, but during any movement spasm can always be found in some muscles and this spasm may pass rapidly from one group of muscles to the other. The tone of all the body musculature is normal during sleep. Due to strong extensor spasm and the asymmetric tonic neck reflex, an athetoid child may be unable to roll over. He may learn to get around on the floor by using his head as lever, while supine in an extended position. In the same way he will find crawling very difficult. Some athetoid children have learnt to walk independently, but are unable to roll over in bed or to crawl. Often the use of the legs is better than that of the arms and some severely affected athetoid have learnt to write, paint and use a typewriter with the feet.


Causes

Athetoid cerebral palsy almost invariably follows an abnormality in the Peri-natal period, and cases divide themselves into two types - those following neonatal jaundice and those following neonatal hypoxia. The cases following neonatal jaundice may be due to maternal rhesus or ABO incompatibility, or to glucose - 6 phosphate dehydrogenase insufficiencies. A cause for neonatal jaundice in the past was enthusiastic over dosage with vitamin K to prevent hemorrhagic disease of the newborn. A high proportion of the cases due to neonatal hypoxia follow a premature birth. There is also a higher than normal incidence of twins and of breech, forceps and Caesarian births. In nearly every case of Athetosis there is a history of severe neonatal disturbance. There does not appear to be evidence of a genetic or intrauterine developmental factor in Athetosis.
Some cases which resemble Athetosis, but have not the same abnormal reflex pattern, may follow encephalitis or a period of anoxia in later infancy. Other children found to be suffering from a form of choreo-athetoid movement defect may later deteriorate and fall into one of the categories of deteriorating neurological conditions.


Early Identification
In babies who will later turn out to be cases of Athetosis, there is almost invariably some concern from the neonatal period. Almost without exception these children are difficult to feed. They may have been unable to suck and breastfeeding may have been discontinued for that reason. Bottle feeding may also have been difficult, and the mother gives a history of feeding taking an abnormally long time. Chewing and swallowing difficulties lead to problems or weaning on to solid food. In some children, the early difficulties may have been so great that they required tube feeding. Even for a short period this is ominous for later motor speech difficulties. All athetoid children show delay in head control. The head is unduly floppy. Hypotonia may last for a considerable time and is associated with marked hypotonia of the whole body. Hypotonia lasting into the second year may suggest an amyotonic condition; but usually the abnormal reflex pattern, particularly of the asymmetric tonic reflex, takes over and fluctuating spasm and tone are found.

In other athetoid children the early poor head control develops fairly rapidly into an extensor spasm. The mother may have difficulty in feeding the child, as whenever she attempts to feed, the child passes into an extensor spasm. Meningitis has been diagnosed erroneously when the child had superadded pyrexia. The mother may notice that the athetoid child never sucks his thumb and is unable to feed himself with a biscuits or a spoon, and never puts toys in his mouth. A study of the child shows that this is due to the retention of the asymmetric tonic neck reflex. On the whole it is found that the hypotonic athetoid child follows brain pathology due to neonatal jaundice: and the hypertonic athetoid follows brain damage due to neonatal hypoxia.

Associated Factors

Speech: All athetoid have a very mild, mild or severe speech defect. It is a dysarthria and may be considered as due to Athetosis of the movement of respiration, swallowing, chewing, phonation and voice production.

Hearing: Following neonatal jaundice and some cases of neonatal hypoxia, there is a strong likelihood of a partial hearing defect which takes the form of a high frequency loss.

A severe hearing loss may be associated with a mild motor handicap, and vice versa.

The hearing defect is due to damage in the nerve pathways presumably at the site of the cochlear nuclei. Some athetoid children appear to have more difficulty in understanding that can be accounted for by the hearing loss. There may be and element of auditory imperceptions (receptive aphasia). The expressive language may be limited in vocabulary but this can be accounted for by the efforts an intelligent child may make to spare words and makes her speech handicap less obvious.

Visual: A high incidence of hypermetropia has been noted in Athetosis. All children should have an eye refraction examination early. In coordinated eye movements occur in nearly every child and many have difficult closing their eyes voluntarily and looking upwards on command.

Epilepsy: This occurs less frequently in athetoid children. If present, it may be of the nature of petit mal attacks. It is presumed that the cerebral defect is at the level of the basal ganglia; and occurrence of true grand mal in these cases suggests additional cerebral cortical involvement.

Intelligence: It cannot be stressed too strongly that these children will very likely have the intelligence that nature intended for them before the cerebral insult. As the cortex is not involved in pure cases of Athetosis the child as regards intellectual deficit is, so to speak, innocent unless proved guilty. In fact quite a number of athetoid have a higher intelligence and have done better educationally, than their siblings. The defect of the inhibitory mechanism which causes the lack of control of movements may in some way liberate the intelligence. It is in this group of cerebral palsy cases that even when they are severely physical handicapped, there may be high academic

For this reason all athetoid children should be assessed for education in a school setting, and no decisions made on isolated IQ examinations. Athetoid do not appear to suffer from the visuospatial difficulties as noted with other groups of cerebral palsy. The incidence of educational sub normality or severe mental sub normality may be comparatively low.


Athetoid Quadriplegia

Many athetoid children present the picture of a "floppy infant" to begin with. Postural tone against gravity is very low. The child is passive and flaccid and there is little spontaneous movement. There are great feeding problems and often abnormal respiration. Bronchitis and bronchopneumonia are frequent occurrences as the child may be too weak even to cough. His mouth is loosely open, his grasp weak or non-existent. His head is turned to one preferred side, often the right. His hands are open with flexed wrists and elbows. There is usually pronounced asymmetry of the trunk. In some children, the Gallant reflex may be present and strong for a long time, even for years, and sometime can be elicited on only one side. Head control is usually absent altogether when pulling the child to sit, in sitting supported, and in prone- lying. The child dislikes prone-lying as he cannot lift his head and look around. Sometimes, in this position, breathing is difficult for him, with the face down and his inability to turn to one side. His legs often show an exaggerated primitive flexor pattern; they are excessively abducted. The feet are dorsiflexed and pronated. It is possible to bend the toes to his shin for many months or even for years; this can be done with normal babies but only for a few weeks. Extension of the legs is weak and incomplete. The legs are rather passive, though one leg, usually the right, may move more than the left. There may be weak reciprocal but no simultaneous kicking. With extension of the leg there is some adduction but no internal rotation at the hips. The typical athetoid movements with their abnormal co-ordination do not develop until later, often not until 18 months or even 2 or 3 years of age. They seem to develop to when the child becomes more active and tries with effort to respond to external stimulation.


Impact of Early Intervention

These children can be diagnosed early, and the service of expert professionals should be available. The value of early treatment in Athetosis cannot be overestimated. The developmental therapist can help with the inhibition of abnormal reflex movement and the development of normal patterns. She/ He can help the child to move around earlier than he would without treatment. She / He can also help with the use of his hands. He will explore his own body and his surroundings earlier if he gets help: and this extra time gained for learning in infancy must affect his later intellectual development. The speech therapist can help with feeding problems and encourage the development of executive language and inner thought.
If possible these children should attend a nursery school for special children at 3 years of age, so that the various methods of therapy can be coordinated and there can be an ongoing assessment of their intellectual ability.
While treatment continues during school hours, he must be allowed the chance of a normal education. Reading is often achieved at a normal age if individual teaching is given. If he cannot write, gadgets such as magnetic letters or a thick pencil must be provided. Computers / Electric typewriters should be available at the time when he should normally be writing. The child's education must include a normal interest in art, music, history, nature study and science.
After help at a special school with education and therapy combined, many of these children may make such progress that they later can take academic examinations such as the matriculation and graduation levels. They will need appliances such as computers / electric typewriter.

TREATMENT

Comparing with other varieties of Cerebral palsy, the management of children with Dystonia is very challenging. The role of oral medication is very important. Traditional therapies do not work so much. The management technique requires a complete team. Parents should be educated about the fluctuation of tone and its complications.

Standard Techniques
a. Handling, Transfer and Carrying Technique
The first and foremost thing to learn is handling, transfer and carrying techniques. Handling means posturing of the child for different needs. There should be emphasis to stimulate the affected side as much as possible.
Transfer means changing of postures. There child should be encouraged to transfer with the help of affected side
Carrying means change of posture and place both. While carrying the child should be given opportunity to use the affected side as much as possible.

b. Use of Aids and Appliances
Postural Aids
In the initial stage, a modified corner chair can help the child to midline orientation with trunk stability. The children with good head control can use the modified arm chair with tray cut out. In all chairs a pommel should be placed to prevent hip dislocation. In rare cases, standing frames are used.
Orthotic Aids
Generally Solid AFO is prescribed for putting optimal weight bearing on feet. Anti-torsion splint may be used to prevent tibial torsion.  
Mobility Aids
Due to fluctuate tone of the child, posterior or anterior rollator are not prescribed. A special training walker is available for these children.
Adaptive Aids
Adaptive aids to promote hand function are being used according to the need of the children

c. Physiotherapy
In the case of Dyskinesia, the role of physiotherapy is very challenging. Tone management is the key factor of the program. Range of motion is not affected but the desired movement with the joint is limited.   

d. Developmental Therapy
Developmental therapy has a great role in case of CP Dyskinesia. Due to fluctuate tone and presence of primitive reflexes (Especially ATNR), the child is unable to gain milestones as other form of cerebral palsy. The child takes more time to achieve head control and sitting.  Unlike other forms of cerebral palsy, children with dyskinesia may be able to walk independently by 15 years age.

e. Occupational Therapy
Hand function achievement is another challenging job with the children with Dyskinesia.
In the initial stage, midline orientation should be prime concern of the occupational therapist. With the help of Adaptive / Assistive aids, the child should be trained to be independent in ADLs.
 
f. Special Education
Mostly children with dyskinesia are intelligent but they can’t communicate effectively due t their speech problems. The special educator should work towards academic with alternative means.
 
g. Speech Therapy
Mostly children with dyskinesia have poor speech so there should be one speech therapist in the management team

h. Role of Oral Medicines
A number of medications have been used in the management of dystonia, including anti-cholinergic medications (e.g. trihexiphenidyl), tetrabenazine, benzodiazepines (e.g. diazepam) and baclofen.
In case of associated Epilepsy and drooling, medication is also used.

i. Role of Botulinum
Botox has a definite role in the management of this category of children. The treatment protocol is same as other Spastic variety.
   
j. Orthopedic Surgery
The occurrence of hip subluxation or dislocation is very high in this category. So the orthopedic surgeon should be included in the team for hip surveillance. In some cases, the child may need surgery for the equinus or flexed knees.

K. Intrathecal Baclofen
This is one technique which can do miracle in selective cases of Dyskinetic CP. In case of good intelligence and motivation, a child with this category can be benefited with ITBP. Earlier only big size pump were available but these days 20 ml smaller size pumps are also available.

L. Deep Brain Stimulation
Apart from ITBP, these days DBS is also available. It is a very sophisticated technique where the neurosurgeon puts a device in the brain with neurosurgical procedure. Although, all cases are not right for the procedure but at large it is helpful. 

Experimental Therapies
A. Hyperbaric Oxygen Therapy
As HBOT is a generalized treatment for children with cerebral palsy, Children with Ataxic CP are also eligible for this treatment system. When HBOT is accompanied with good pediatric therapy, encouraging results have been observed. Isolated HBOT has no role in the total management of a child with Dyskinetic CP.

B. Stem Cell Therapy (SCT)
Stem cells are considered repairing cell of the body. As the children with CP Ataxia have insulted brain, stem cell can play a role in the management. But there is no concrete data available for the same. Researches are going on, Hope for the best! 

Exploratory Therapies
A large number of parents have reported some results from these therapies so these can be associated in the total management program. Isolated use of these therapies has very little role. So whenever, you start any of therapies, please continue the standard therapies as usual.

  1. Homeopathy
  2. Ayurveda
  3. Unani
  4. Acupuncture
  5. Acupressure
  6. Hippo-therapy
  7. Hydrotherapy 

Prognosis

It was found that about 50% of cases walked before 5 years, and a further 20 - 25% before 11 years. Some cases achieve independent walking in late adolescence, may be at 15 years of age.

Employment
Because of poor hand control, these intelligent cerebral palsied people will not be good workers in workshops or on factory benches. It is a tragedy to see potentially intelligent handicapped people doing the very job they are worst at. Their education should be aimed at more intellectual pursuits such as typewriting, proof -reading, computer programming etc. Because of the severe physical disability many athetoid cannot be gainfully employed. If they have educational skills such as reading they will have much greater insight into their problems and will find it easier to adjust. There have been many surprises, in every case because somebody had the faith to continue with an academic education. Some of these people have shown the maturity to make a happy marriage with another similarly disable person. The whole of this prognosis depends on early diagnosis of the condition of Athetosis and the determination of professionals involved giving them the best environment for full development.


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