(Infantile hemiplegia)

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.

When the child makes a movement with his non - affected limb, the affected side partially or completely assumes the typical hemiplegic position.

These associated movements are primitive reflex movements, because the limb is no longer under cortical control, and are postural reactions allied to tonic reflexes and decerebrate rigidity. These associated movements might be a serious handicap to the child.

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.

With opportunity and stimulation all hemiplegic children should walk, though the passing of this milestone may be delayed. They should with training, learn to use the affected hand as a helping hand at table and when opening doors and using machines. The vast majority has speech, the majority at the normal time. Hemiplegic children may have specific perceptual difficulties; and these were more in evidence in left hemiplegia than right. All hemiplegic children should be watched by an educationist ready to give specific help to the child if it is needed. Cases of acquired hemiplegia appear to have more difficulties than congenital cases.

What is the possibility of employment?

The placement of adult hemiplegic possibly with more organized early help the results could be better. The majority of hemiplegics with / without supervision can work in open market.

What are the general features of CP spastic diplegia?

This is the original condition described by Little in 1856. Many cases follow a premature birth and with very low birth weight.

The incidence of this condition varies according to the early care of methods used to prevent prematurely and of the early care of premature babies.

These children show

• 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.

How early the disability can be diagnosed?

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.

How good or bad the prognosis is?

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.

What are the employment chances?

Employment in later life is influenced by the presence of visuo-perceptual difficulties. If the child is physically disable and educationally below average, and in addition has difficulties in manipulation, recognizing shapes and mastering writing and arithmetic, it may be impossible to find an open job for him. Factory or bench work may be ruled out because of his inability to understand shapes.

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.

There is a vast list of causation in the case of spastic quadriplegia.

Prenatal causes

These conditions are considered as high risk. Mother with thyroid disorders Mother with mental retardation Mother with seizure disorder A lot of studies are going on to justify the high risk factors. The specific causes of CP spastic quadriplegic include developmental brain abnormalities, genetic or chromosomal abnormalities, radiation exposure, prenatal infections, illicit drug use and fetal alcohol syndrome.

Perinatal causes

Birth asphyxia ( Hypoxic ischemic encephalopathy), Birth trauma, etc.

Postnatal causes

Meningitis, traumatic brain injury, encephalitis,

How early the diagnosis can be made?

Spastic quadriplegia 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 in 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.

What are the additional factors 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.

How a Child with severe spastic quadriplegia differ from a child with less affected spastic quadriplegia?

1. The severely affected children

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.