CEREBRAL PALSY SPASTIC HEMIPLEGIA or Unilateral Spastic CP (Infantile hemiplegia)


General Features
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.

Associated movement
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.

Different types
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.

Common causes
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.

Early Identification
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.

Associate factors
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.

TREATMENT

The word management is always better than treatment in the case of cerebral palsy. There are three types of management techniques are available for children with cerebral spastic hemiplegia. They are Standard, Experimental and Exploratory techniques.

Standard Therapies
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
The children with spastic hemiplegia may be helped with Corner chair, Arm chair with tray cut out, Standing frame, etc according the need of these children. Very few cases require the help of standing frame as these children have good trunk.

Orthotic Aids
These children should be placed in AFO early as preventive care during night. When AFO is prescribed for the affected foot, the other foot is taken care with foot plate. Otherwise, the child will have leg length discrepancy in the long run. AS the upper limb is more affected, Modified Cock up splint should be used to keep the wrist and thumb in neutral position as night splint. The hand splint if it is static should be used during day rather CIMT band should be used for the normal hand. A large proportion of this group has Genu recurvatum so modified angular AFO should be tried. In case of Genu recurvatum, gaiters have no role at all. 
 
Mobility Aids
Very few children of this group require the support of mobility aids.

c. Physiotherapy
The main components of the PT program should have these elements.
Alignment Program
Stretching Program
Strength Training Program

d. Developmental Therapy
Milestones achievement Program to achieve
Gross motor Skills
Mobility skills
e. Occupational Therapy
Milestones achievement Program to achieve
Fine motor skills
(Both Bimanual and CIMT based)
Independence in Activities of daily living
Sensory Integration (In case of Sensory dysfunction)

f. Special Education
Preparation for inclusion in regular school set up
To reduce perceptual problem if any
(Mostly children with Hemiplegia do not require special education)

g. Speech Therapy
Language development
Communication skills
(Very few children with Hemiplegia require speech therapy)

h. Role of Oral Medicines
Generally medicines are required for Epilepsy, and drooling management. In rare cases, antispastic drugs like Baclofen, Tizanidine are prescribed. Due to unilateral involvement, generalized antispastic drugs are controversial to use.

i. Role of Chemodenervation
Generally Chemodenervation is done with phenol or Botulinum toxin type A (Botox). Botox is considered better than phenol nerve block. Botox can be injected as young as 2 years old. The golden period for Botox is 2-8 years of age. Botox can be given after 8 years of age also, when the child is not a right candidate for orthopedic surgery. The targeted muscles are from lower limb and upper limb both. In the case of lower limb, Gastroc, Soleus, Tibialis posterior, Medial Hamstrings, Hip Adductors (Rarely Hip Flexors, Hip internal rotators) are injected. In the upper limb, Pronators, Biceps, Brachoradialis, Flexor carpi radialis, Flexor carpi ulnaris, Flxor pollicis longus, Adductor pollicis, Flexor pollicis brevis / opponens,  Flexor digitorium profundus,  Flexor digitorium superficialis are injected.
Always remember, the result of Chemodenervation depends on Post Phenol / Botox therapy. As post Phenol / Botox therapy is not available in most of the physiotherapy centre, the role of Botox / Phenol has been underestimated.  
  
j. Orthopedic Surgery
Orthopedic surgery has definitive role in the management, especially for true equinus, knee flexion, adducted and internally rotated hip. The best timing of the surgery is before emerging compensatory toe walking with the unaffected limb. Hip subluxation is one of the risk factors with leg length discrepancy. Scoliosis has been seen with long term toe walking. If the child requires upper limb surgery, it should be done before the child start ignoring the hand completely.     
A large number of children with spastic hemiplegia have Genu recurvatum (Hyper-extended knee) also. These children should be evaluated very carefully before surgery.  
 

k. Rhizotomy
This group of children generally does not require SDR.

l. Intrathecal Baclofen
As these children have more spasticity in the upper limb comparing the lower limb so the role of ITBP is not so high.

Experimental Therapies
A. Hyperbaric Oxygen Therapy (HBOT)
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 Spastic Hemiplegic 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. Hydrotherapy has been considered one of the best alternative therapies to encourage spinal / Truncal control and balance.
  2. Ayurveda
  3. Unani
  4. Acupuncture
  5. Acupressure
  6. Hippo-therapy
  7. Homeopathy

Prognosis
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.

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.

Types of Cerebral Palsy

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