Welcome to ICD, centre for specialized treatment in Cerebral Palsy

The definition and classification of cerebral of cerebral palsy have always been a source of great confusion and controversy. The term itself is somewhat misleading: whereas, CEREBRAL appropriately emphasizes the importance of the brain, PALSY is an anachronism that in modern parlance is usually associated with paralysis which suggests a complete loss of movement (not typical of cerebral palsy).

There are so many definition of cerebral palsy; there are three recurrent elements that provide the basis for a concise definition.

There is a significant problem with motor function.
This motor impairment results from something that went wrong with the brain during its early development.
The disturbance of typical brain development occurred over a discrete period of time and does not represent a continuing, recurrent or progressive process. So cerebral palsy is considered as;

Infantile hemiplegia is a movement defect of one side of the body only, either the right side or the left. The disability is not always uniform. In the typical spastic form in the older child, the following patterns are seen.

the upper limb is held with the arm adducted and internally rotated,
the forearm flexed and pronated,
the wrists flexed,
and the fingers flexed
with the thumb pressed into the palm.

Unless, the child is helped to use the arm, contracture develops in
the elbow, wrist flexors and the forearm pronators. The arm becomes fixed and useless in that position.

There is often little abnormality of hip movements and adductor spasm is not usually noted.
There is severe spasticity of the calf muscles and the foot is held in plantar flexion.

Dorsiflexors of the foot is always weak in hemiplegia.

Unless, practiced very adequately from an early age, the spasm in the calf muscles may lead to permanent contractures.

Because of poor dorsiflexion, the child tends to walk with his heel raised on the affected side and to compensate, abducts the hip and swings the lower limb or hyper extends the knee.

Dorsiflexion of the foot is easier to perform with the knee flexed. In severe cases, there is spasm in the trunk muscles of the affected side. This causes a scoliosis and by raising the pelvis produces an apparent shortening of the lower limb, which in turn causes further plantar flexion.

The lower part of the face and tongue may be affected and there may be an internal strabismus on the affected side.

At least four other types of hemiplegia are seen.

A hemiplegic child may show hemiathetosis. There are typical athetoid movements on one side only. In these cases, the girth of the affected limb may be increased. Cases may occur where the defect may be predominantly athetoid in the arm and in the leg spastic
A hemiplegic child may show marked sensory loss, with spasticity present often to a minimal extent. The sensory loss takes the form of an astereognosis and in some cases there is a loss of sense of position. The disorder of mobility may be due to apraxia and not be spasticity.
Spasticity is minimal, there may be no evidence of astereognosis and yet the child refuses to use his affected arm. He may attempt very awkwardly to button his clothes with his non-affected hand making no attempt to help with is affected hand. Yet, there appears to be no obvious reason why he should not use it. The child may have a defective body image and may forget about his affected hand.
Minimal spasticity and often no sensory loss, but a pronounced defect in growth.

About two-thirds of the cases of hemiplegia date from birth and about one-third follow an illness catastrophe in infancy.

1. CONGENITAL

2. ACQUIRED

The congenital causes can be described under three headings:

1. CONGENITAL

a. SW Syndrome:

The condition has a naevus on one side of the face and evidence of a naevoid condition in the brain, causing a hemiplegia of the opposite side. There is usually intra-cranial calcification. Mostly these children suffer from epilepsy and mental retardation.

b. Absent Corpus Callosum:

This congenital abnormality may cause a hemiplegia.

Other developmental abnormalities, such as hydrocephalus, may be associated with hemiplegia.

c. Birth Abnormalities

The majority of children with congenital hemiplegia are born after an abnormal pregnancy or birth process. Pre-eclampsia, pre-maturity, post-maturity, or abnormal delivery such as forceps, breech, twin or Caesarian birth may be a factor.

d. No Apparent Cause

In about 20% of cases, no cause can be found for the condition. There may have been unsuspected cerebral involvement during birth or a cerebro-vascular incident.

3. ACQUIRED

During the last few years, we have seen a large number of cases with acquired hemiplegia. There are numerous causes for the development of hemiplegia during early life in a previously healthy child.

1. Hemiplegia may follow an attack of childhood infective illness such as pertussis, measles, mumps or chickenpox. Damage may follow an infantile convulsion associated with the illness, or the underlying pathology may be a vascular lesion or encephalitis.

2. Hemiplegia may follow immunization, the immediate cause being a fit occurring within hours of the injection.

3. It may be due to a cerebral venous thrombosis during pneumonia, meningitis, following surgery such as appendectomy and more rarely now, tuberculoses meningitis. It can occur after severe dehydration in gastroenteritis.

4. Hemiplegia has followed arterial thrombosis in a congenital heart defect such as Fallot's tetra logy, or in sickle cell anemia.

5. Possibly the commonest presentation is of sudden onset in a previously well child. He convulses and passes into coma. On recovery he is found to have a permanent hemiplegia. This type of hemiplegia has been the subject of a booklet in the Little Club Series. The author produces evidence that the cause of this condition is a vascular anomaly. There may be arterial or venous aneurysm, or from an angioma. There may be arterial occlusion due to focal arterial or venous thrombosis. There may be trauma to the internal carotid artery. These conditions are revealed by angiographies.

6. Occasionally hemiplegia develops slowly and insidiously and must be part of a deteriorating neurological condition. A cerebral tumor must be considered.

7. Hemiplegia may develop after a prolonged convulsion or status epileptic us in a known epileptic child. The possibility of this catastrophe makes it imperative, that at all times active measures are taken to prevent any epileptic fit lasting longer than 15 to 20 minutes.

8. Hemiplegia may follow injury such as a road accident or baby battering. There may be a subdural haematoma that required immediate evacuation.

The defect is usually noticed about the sixth month of life or later.
There may have been asymmetry in the child's early movements, and persistence of the asymmetric tonic neck reflex to one side. Some parents have noted early clenching of the fist, but in many cases nothing abnormal is seen until cortical control of movement takes over from the infantile reflex patterns. The parents may notice that the child is disinclined to use one hand and the affected hand may be still. The defect in the arms is usually noticed before that of the leg.

Children with hemiplegia may be late in sitting up or in crawling often they shuffle on their bottoms rather than crawl. Walking may also be late. The majority of cases are noted in the first year of life, but occasionally a very mild hemiplegia is only noted when another handicap, such as epilepsy or educational sub normality, is being investigated.

Hemiplegia is often recognized quite early in fact much earlier than diplegia, because of the obvious asymmetry of the child's postures and movements. A few children are already referred for treatment at 5 months of age. More often, however, they are diagnosed at 8 or 9 months of age or later, when it is noticed that they cannot sit up and when they reach and grasp only with one hand. Some asymmetry of posture is still normal up to 4 months of age and a diagnosis is therefore difficult unless the hemiplegia is of a severe degree. Furthermore, initially the baby is often referred for treatment with a diagnosis of Monoplegia because of constant fisting of the affected hand, while the involved leg appears to be normal.

Visual Defects: In about one - third of cases of infantile hemiplegia there is a field of vision defect. It is a homonymous hemianopia affecting the field of vision on the side of the affected limb. Strabismus, due to a refractive error or to the field of vision defect or to underlying optic atrophy on the affected side, may be present.

Hearing defects: Hearing defects are not common

Sensory Loss: There may be astereognosis of the affected hand. Blindfold, the child may not be able to distinguish a marble, cotton wool or penny in the affected hand, although he can do so readily in the other hand. This blurring of sensation may be a factor in the child's refusal to use his hand. It may be associated with a hypotonic hand and arm in which there is defective growth, rather that a still spastic limb. Other hemiplegic children may have a body image defect on the affected side, and this prevents use of the affected hand.

Growth: The affected limbs may be thinner and shorter than those on the normal side. This may not be due to lack of use or spasticity but appears to be cerebral in origin. Shorting of the leg may further increase planter flexion.

Speech: The motor control of speech is usually normal in hemiplegia unless there is bilateral involvement of the face and muscles used for speech. This occurs in occasional case of congenital suprabulbar palsy. Hemiplegic children may have delayed language development due to mental retardation; but there may be specific dysphasia due to damage to the speech centers in the affected hemisphere.

Epilepsy: Epileptic attacks occur very commonly. The fits may date from birth or occur for the first time in infancy, childhood or adolescence. There may be frequent fits or an occasional episode. Onset of epilepsy before three years appears to have a poor prognosis for later intelligence.

Intelligence: As an average it can be said that the brain damage associated with infantile hemiplegia depresses the child's intelligence quotient by 20 points. It was found that the intelligence in left hemiplegia was lower than in right hemiplegics. There is a preponderance of cases of left hemiplegia over right in many hospitals for the severely subnormal and of right over left in the general population.

Before a prognosis about schooling and employment is given to parents of a hemiplegic child, a psychologist's opinion should always be obtained. Entirely erroneous advice may be given to the parents unless the child has had a detailed psychological assessment.

Behavior Problems : Hemiplegic children show the expected behaviors problems of disable children trying to complete with their peers; attention seeking behavior, temper tantrums, immaturity and overdependence. The parents need help to deal with these problems. The specific behavior problems which hemiplegic may show are hyperactivity and sudden unexplained aggressiveness. This is nearly always associated with an abnormal EEG record and with epilepsy. There may be a temporal lobe focus.

symmetrical or near symmetrical involvement
the legs are more involved that the arms.
In the erect or supine position the child shows the typical scissor pattern which is associated with the condition.
The head and back are held in an extended position, the hips are extended internally rotated and adducted, the knees are extended and the ankles are in the plantar flexion position.
It is extremely important, when examining these children, to look at the movement patterns in different postures.
If the child is placed in the sitting position, the head may be flexed, the back may assume an infantile totally flexed curve.
The hips knees and ankles may flex and the child can sit in the tailor position on the base of his spine.
If he is placed prone his head may rise from the couch and his body extended with extended hips, Knees and planter flexion of feet.
If he is maintained in an all fours crawling position, the head, hips, knees and ankles may flex, and he may show spasm in dorsiflexion rather than in planter flexion.
If the head is raised in this position, the hips and knees may extend.
In a less involved child, the primitive tonic neck reflex may take over and he will actually flex the hips and move in a symmetric bunny or kangaroo hop.
The increased extensor spasm prevents the child from rolling over and the absence of body-righting reflexes prevents him from developing a reciprocal flexion and extension of the hips and knees to rollover smoothly. In the same way a smooth crawling pattern is impossible.
The arms may be mildly, moderately or severely affected.
If they are entirely normal, the condition can be called a spastic paraplegia.
Usually there is some involvement of the upper limbs. If the arms are moderately affected, one hand is better that the other because the child will use one hand for play. The fingers may be spastic and clenched. In attempting to hold an object, band may show what might be called an avoiding reaction and the fingers are hyper-extended. In some children there is marked hyperextension of the first and middle phalangeal joints but acute flexion of the terminal phalangeal joints.
In more severely involved children the arms are retracted at the shoulders in extension and the child finds difficulty in bringing his arms down to his side when lying spine. Both hands are severely involved and he finds difficulty in feeding himself and playing with toys. If a child with diplegia is maintained in one position for the greater part of the day, particularly if it is a flexed posture, he may develop flexion contractures in muscles and in the capsules of joints.
Severe deformities and joint dislocations can result.
In these severe cases the term spastic quadriplegia may be used. It is sometimes difficult to destining between spastic quadriplegia and bilateral hemiplegia, particulars in the older child when contractures and possibly dislocation of the hips have occurred.
Very mild cases of spastic diplegia may involve the legs and feet only. There may be a tendency to walk in planter flexion only. In some cases the condition appears to be a Monoplegia.

In most cases the baby is premature by dates and some children are born as much as 12 weeks before they are expected. Many have very low birth weight; under three pounds and some fewer than two pounds. In some cases the child is a twin or one of a triple. There may be a maternal history of previous miscarriages or premature births and the birth suggests some form of maternal inadequacy. Other premature births resulting in spastic diplegia have followed antenatal hemorrhages in early pregnancy and also pre-eclampsia. Spastic diplegia may follow a full term abnormal birth, or a breech, Caesarian or forceps delivery. In the majority of cases the child was severely ill in the neonatal period.

A form of spastic diplegia, identical to the prematurely born cases, may occur in more that one sibling.

In some diplegia cases there is no abnormality or family history to account for the condition. In these cases there must be a strong likelihood of a recessive condition. The danger of another child being affected is 1: 4. This type of case must be borne in mind when giving genetic advice. In several cases following a premature or abnormal birth, the incidence of similarly affected members of the family suggested that the birth history was irrelevant.

Congenital anomalies have been noted in association with spastic diplegia. Researchers mentioned talipes, congenital heart defect and congenital dislocation of the hip. Few researchers have seen cases with cleft palate, and with absent corpus Callosum.

Spastic diplegia usually follows an abnormal birth, and in many centers the children are on an 'at risk' register. Many have been carefully followed up and observed. Some cases are not obviously affected in the early months of life. They may show normal walking and placing reflexes in the neonatal period and reciprocal kicking in infancy. The Moro reflex and the asymmetric tonic neck reflex may be longer in evidence than is normal.

The defect is usually noted when the child is late in rolling over and in sitting up. He is late in developing the balance reflexes in sitting, crawling and standing.

Many of these babies are premature and their slow development is thought to be due to this. Their milestones are delayed but postural tone is fairly normal early on. The physiological excess of flexor tone seen in very young normal babies may remain unaltered for many months. Although later than normal, head control develops and the child's arms and hands seem to be unaffected. He can get his hands together in midline and to his mouth and he develops midline orientation of his head. His legs shown little spasticity initially; they flexed and abducted, though full passive abduction may be somewhat resisted. For these reasons, diagnosis is not usually made before about 9 months of age when the child dose not sit up by him and has no balance when made to sit. Some mildly affected children may not be diagnosed until 18 months or even 2 years of age, when they pull themselves, up to standing and start walking on their toes. Then they usually show an asymmetrical pattern of standing and walking on one foot on the toes with little weight on that leg, the other foot heel down but with hyper -extension of the knee and flexion of that hip.

Speech: The more severely involved cases of spastic diplegia show a dysarthria. There is impairment of voluntary movement of the lips, tongue and palate, and the child may dribble. The tongue may be stiff in its movements. Speech is low and labored.

Hearing: Hearing may be affected, particularly if there has been neonatal asphyxia or jaundice of pre maturity.

Visual: The majority of these children, even those mildly affected have in coordination of eye movement. There is typically an internal strabismus, but many children have difficulty in holding their eyes for a period on a desired task. Myopia and cataracts are likely additional handicaps in a prematurely born child.

Perceptual: Prematurely born children in particular show the visuo-motor and perceptual defects. Before a child with spastic diplegia is allowed to attend a class with normal children, the possibility of a perceptual defect must be checked. If it is not noted, the child may have extreme difficulty with normal class work.

Epilepsy: About a third of the children with spastic diplegia have an epileptic fit once or twice in their childhood, often associated with an infective illness.

Intelligence: The level of intelligence in spastic diplegia has some relationship to the severity of the disability. A fairly high proportion of the mild cases - possibly 50% - have an intelligence within the normal range; and some have above average ability. Visuomotor and perceptual difficulties occur frequently in this condition, and affect educational achievement. In the more affected cases, where there is severe motor involvement of the hands and additional visual and learning problems. It is impossible to suggest a numerical figure for the intelligence quotient. With individual education they may achieve reading and a high level of general knowledge. The visuo-perceptual problems will prevent an overall good educational attainment. Some are definitely below the level able to benefit from formal education, but there are many surprises. In this group, there are many who university graduate are.

These children should be diagnosed early and treated as babies on neurological lines. The result of early treatment can be surprising. Eventual independent walking should be achieved in all cases where the use of hands is nearly normal. Other children with more severe involvement of hands should walk with the use of elbow crutches. Few of them may later need to use a wheelchair or motorized vehicle; but enough mobility should be achieved in trunk and leg movements for the older child to get out of his chair independently for everyday living activities. If the child can walk independently early in school life, he should be able to manage in a normal class at school. Others, due to the need for treatment and special chairs and appliances during school life, may need education in a special class.

There are 40% - 45% children are spastic quadriplegic in the total prevalence of those with spastic cerebral palsy, all four limbs and usually the trunk and muscles that control the mouth, tongue and pharynx are affected. The severity of the motor impairment in Spastic Quadriplegic implies under wider cerebral dysfunction.

These children show symmetrical or near symmetrical involvement; and the arms are more involved than the legs.
In the erect or supine position the child shows the typical scissor pattern which is associated with this condition.
The head and back are held either in an extended position.
The hips are mostly flexed and internally rotated and adducted; the knees are either flexed or hyper extended with plantar flexed ankles.
It is extremely important when examining these children to look at the movement patterns in different postures.
If the child is paced in the sitting position, the head may be flexed, the back may assume and infantile totally flexed curve, the hips, knees and ankle may flex, and the child can sit in the couch position on the base of his spine.
If he is placed prone, his head may rise from the couch and his body extended with extended hips, knees and plantar flexion of feel.
If he is maintained on all fours crawling position, the head, hips, knees and ankles may flex and he may show spasm in dorsiflexion rather than in plantar flexion. If the head is raised in this position, the hips and knees may extend.
In a less involved child, the primitive tonic neck reflex may take over and he will actually flex the hips and move in a symmetric bunny or kangaroo hogs.
The increased extensor spasm prevents the child from rolling over, and the absence of body righting reflexes prevent him from developing a reciprocal flexion and extension of the hips and knees to roll over smoothly. In the same way, a smooth crawling pattern is impossible.
The arms may be mildly, moderately or severely affected. If the arms are moderately affected, one hand is better than the other because the child will use one hand for play. The fingers may be spastic and clenched. If attempt to hold an object, hands may show what may be called an avoiding reaction and the fingers are hyper extended.
In some children, there is marked hyperextension of the first and middle phalangeal joints but acute flexion of the terminal phalangeal joints.
In more severely involved children the arms are retracted and the shoulders are in extension and the child finds difficulty in bringing his arm down to his side when lying supine. Both hands are severely involved and he finds difficulty in feeding himself and playing with toys.
If a child with spastic quadriplegia is maintained in one position for the greater part of the day, particularly if it is a flexed posture, he may develop flexion contracture in muscles and in the capsules of joints. Severe deformities and joints dislocations can result.

What are the common causes associated with spastic quadriplegia?

Speech: The more severely involved cases of spastic quadriplegia show a dysarthria. There is impairment of voluntary movement of the lips, tongue and palate, and the child may dribble. The tongue may be stiff in its movements. Speech is low and labored.

Hearing: Hearing may be affected, particularly if there has been neonatal asphyxia or jaundice of pre maturity.

Visual: The majority of these children, even those mildly affected have problems with co-ordination of eye movement. There is typically an internal strabismus, but many children have difficulty in holding their eyes for a period on a desired task. Myopia and cataracts are likely additional handicaps in a prematurely born child.

Perceptual: Prematurely born children in particular show the visuo-motor and perceptual defects. Before a child with spastic quadriplegia is allowed to attend school normal or special school, the possibility of a perceptual defect must be checked. If it is not noted the child may have extreme difficulty with academics.

Epilepsy : Lot of children with spastic quadriplegia have an epileptic fit once or twice in their childhood, often associated with an infective illness.

Intelligence: The level of intelligence in spastic quadriplegia has some relationship to the severity of the handicap. A fairly high proportion of the mild cases - possibly 50% - have intelligence within the normal range; and some have above average ability. Visuo-motor and perceptual difficulties occur frequently in this condition, and affect educational achievement. In the more affected cases, where there is severe motor involvement of the hands and additional visual and learning problems. It is impossible to suggest a numerical figure for the intelligence quotient. With individual education they may achieve reading and a high level of general knowledge. The visuo-perceptual problems will prevent an overall good educational attainment. Some are definitely below the level able to benefit from formal education, but there are many surprises.

In these children, the prognosis is bad, even when they are treated early in life as spasticity or rigidity is already strong at a few weeks or months of age. Epilepsy may be present early or develop later on. Fits may be of all kinds: myoclonic jerks, petit mal, or major convulsions. May be associated with a child's physical handicap. In some children, there are visual defects, partial or total blindness, or visual agnostic. Hearing defects more auditory imperceptions may also be present.

In supine, opisthotonous, together with a complete lack of head control, are early symptoms, as are a rigidly extended spine with shoulder retraction adductor spasticity and excessive extension of the legs. Contractures of the adductors may develop very early on and be equally strong, with flexed or extended legs. In the early stages, there may not yet be internal rotation of the legs at the hips but this will develop later on. To begin with, the ankle may still be dorsiflexed, but they will soon plantiflex, when the child is left lying on his back for most of the day, or when he is put on his feet to try to make him stand. Asymmetrical tonic neck reflexes are usually pronounced, the head preferably turned to one side with lateral flexion of the neck to the opposite side. There may be resistance to turning the child's face to the opposite side. The lateral flexion of the neck will affect the whole spine, resulting in asymmetry of the trunk and obliquity of the pelvis. This is associated with a dysplasia of, usually, the left hip. In some cases, adductor spasticity and internal rotation of the legs may produce dissociation of both hips. When placed in prone-lying, the child cannot lift his head and extend his spine and hips. He may not even be able to turn his head to one side to free his airways, and he will dislike prone - lying because of the difficulty of breathing in this position. His mother, therefore will not him into prone and he will be left lying on his back for many months.

He cannot sit unsupported and falls over to one side. His back is very round, his hips are insufficiently flexed and his legs mare too much addicted to give him a sitting base. This combined with the asymmetry of his spine, may produce a kypho-scoliosis.

Early, and for a long time to come, there are great feeding problems. Tongue -thrusts are frequent as well as difficulties of sucking and swallowing and the child may tend to choke when fed. Some children suffer from reversed breathing, or strider and respiration present problems, especially at night.

Even early treatment may have only limited results in furthering the child's development. However, it can help to prevent some, if not most, of the contractures and deformities, which quickly develop, often in a matter of months. Treatment and advice on home management, especially in the early stages, when we do not yet know what potential the baby may have, are necessary to help and support the family. It helps the mother in handing the child with greater ease and more confidence.

Fortunately, the majority of spastic quadriplegias are not so severely affected as those described above. The milder cases develop spasticity more gradually during the first year of life, although early sign can often be detected at 3-4 months of age, sometime even earlier. Spasticity may not become strong enough to prevent the child from moving. The distribution of spasticity always affects one side more than the other. It may be severe on one side and moderate on the other. It may be moderate on one side and slight on the other. This asymmetrical distribution of spasticity, as well as the exclusive use of one arm and hand only, favors a tendency to scoliosis. Whereas the severely involved child, who cannot move at all , becomes deformed by remaining in a few abnormal postures, the less severely affected child tries to move and function, but can do so only in a few abnormal ways, i.e. in stereotyped abnormal patterns and with excessive effort, which increases his spasticity. Sometimes children who have been diagnosed as spastic quadriplegias at 8 to 10 months of age develop athetoid movements later on, in addition to their spasticity. This usually happens when they become more active around 18 months to 2 tears of age.

Athetosis describes a child whole body passes into a state of slow, writhing muscular contractions when attempting 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.

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.

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.

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.

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.

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.

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.

In this ill-defined group of cases there are so me, where the condition is inherited as an autosomal recessive, and two or more in coordinated children occur in one family. In an occasional family, the condition is dominant and the child has the same neurological abnormality as one of his parents. The motor disability may follow a development abnormality. Children who survive a repair of an occipital encephalocoele may show a true cerebellar ataxia, as part or whole of the cerebellum may be involved in the defect. Several of these children have hydrocephalus due to a congenital abnormality, perinatal brain damage or later cerebral infection. An abnormal birth with severe neonatal difficulties appears to be a factor in the causation of ataxia, as in other movement defects. Forms of ataxia have followed various forms of meningitis or viral encephalitis.

Ataxic children may show all the additional defects of other cerebral palsied children -

Speech: All ataxic have a very mild, mild or severe speech defect.
Due to Hypotonia, the movement of respiration, swallowing, chewing, phonation and voice production can be affected. Hearing: There is a strong likelihood of a partial hearing defect which takes the form of a high frequency loss. Visual: A high incidence of hypermetropia and myopia has been noted in Ataxic. All children should have an eye refraction examination early. In coordinated eye movements occur in nearly every child Epilepsy: This occurs frequently in ataxic children. Intelligence: These children generally have poor cognition due to visuoperceptual problem.

Some workers in the field of cerebral palsy would deny the existence of this group of cases. The world commission on cerebral palsy felt that the condition did exist and defined it. There are children and adults in institutions for the mentally subnormal who are described as 'spastic' by the staff, but who do not fit into the pattern of spastic diplegia or bilateral hemiplegia. They tend to be generally rigid. There is co contraction of both extensors and flexors and a type of lead pipe rigidity when passive movement is attempted. Some of these children are profoundly subnormal and retain this general stiffness throughout life, lying in a flexed posture with no understanding of the world.
Some children show severe retardation and general stiffness, but with physiotherapy or persistent stimulation the rigidity may be overcome and the child later become able to walk and use his hands. This early rigidity is in marked contrast to the Hypotonia shown by some mentally subnormal children.

CP Rigidity children may show all the additional defects of other cerebral palsied children -

Speech: All children with CP Rigidity have a very mild, mild or severe speech defect. Due to rigidity, the movement of respiration, swallowing, chewing, phonation and voice production can be affected.

Hearing: There is very less likelihood of a hearing defect.

Visual: A high incidence of myopia has been noted in children with Cp Rigidity. All children should have an eye refraction examination early. In coordinated eye movements occur in nearly every child Epilepsy: This occurs very less frequently in children with CP Rigidity.

Intelligence: These children generally have good cognition but due to poor hand function they are considered as mentally subnormal.