CEREBRAL PALSY SPASTIC QUADRIPLEGIA or Double Hemiplegia Spastic CP



General Features

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.

Common Causes

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,


Early Identification


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.


Associated Factors

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.

Difference between severe spastic quadriplegia and Mild 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.

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.

2. The quadriplegic child who is less severely affected

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.


TREATMENT

The management / treatment of this group of children is very challenging job. Due to multiple problems, these children require lot of input from professionals and care providers. Generally, the prognosis is not so good but with good and continuous effort most of the children can become independent in life. 

Standard Techniques
a. Handling, Transfer and Carrying Technique
These children require special handling during infancy as these children have hypotonic trunk and hypertonic limbs. Postural alignment should be taken care properly to reduce the chance of hip subluxation scoliosis and other postural deformity.

b. Use of Aids and Appliances
Postural Aids
These children should be provided corner chair with tray cut as they become 3-4 months old. These children take more time to achieve head control and prone development comparing to Spastic Diplegia. Arm chair with tray cut out and pommel should be used to get good trunk control and hand function. Standing frames are also being used in this group.
 
Orthotic Aids
Preventive Orthotic aids should be used to reduce mal-alignment of ankle joints. The occurrence of hip subluxation is very high in this group so special splints should be made. These children may require splints for the upper limbs also as they have elbow flexors, mid arm Pronators and wrist flexors tightness. A few cases may require Thumb abduction splint also. Although, the use of HKAFO / Caliper has been outdated in the management of cerebral palsy, a few children can be benefited with these splints.
 
Mobility Aids
These children should be given support to the trunk while making walker. The actual FMS has been developed for this group of children. Children who are more than 3 years old and not able to move from one place to other with any means should be provided with wheel chair. Professionals should explain the need of the wheel chair clearly and should work simultaneously to make the mobile also.

c. Physiotherapy
These children require intensive physiotherapy to get good joints alignment. Stretching should be done with caution. Muscle strengthening exercises should be practiced more than stretching exercises. The postural muscles should be respected and guarded stretching should be done. Exercises for the Trunk and upper extremity should be designed especially.
 
d. Developmental Therapy
In case of postural development, these children require more time and effort. Prone development should be the basic of all postural stability. The Developmental therapy program should consist of all postural exercises such as static postures, dynamic postures, transitory postures, neuro-enhancing postures and mobility training.

e. Occupational Therapy
As this group of children have problems with upper extremity more than the lower extremity, the role of the occupational therapist is very high. These children should be given regular OT to achieve hand function so that they become independent in ADLs. Hand writing is another area where the occupational therapist has its own place.

f. Special Education
Mostly these children require special education as they are late in achieving independent mobility and good hand function. A few children of this group are very intelligent with very poor trunk so they should be placed in regular schooling if possible.
Special educator should work on alternative mode of communication in case of difficult hand function and speech.

g. Speech Therapy
A speech therapist can play vital role to make a child to be able to communicate. ICF model should be practiced and communication should be first goal in the total management. All children of this group do not require speech therapy.

h. Role of Oral Medicines
Medicines are prescribed for associated problems like epilepsy, poor immunity, drooling, GER, spasticity, etc. Experts feel that this group of children should be tried with anti-spastic drugs. Mostly children with Spastic Quadriplegia have hypotonic trunk so dosage of the medicine should be optimal. 

i. Role of Chemodenervation
A few cases of this group can have focal spasticity also. These children may be helped with Chemodenervation through Botox or Phenol. The upper extremity should be respected with Botox. There is no or little role of phenol nerve block in upper limbs. Phenol nerve block can replace Botox for hip Adductor tightness.

j. Orthopedic Surgery
The first thing should be taken care by the orthopedic surgeon is the hip containment. The chances of hip dislocation, scoliosis is very high in this group of children. Children with spastic quadriplegia may require surgical intervention for ankle, knee, hip and elbow joint also.

 k. Rhizotomy
This group of children is eligible for selective dorsal Rhizotomy due to generalized spasticity. A good surgical procedure can do miracle in reducing tightness in these children.

 

l. Intrathecal Baclofen
Children with good intelligence and severe spasticity are also benefited with Intrathecal Baclofen Therapy. Case selection is very important.

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 Quadriplegia 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.

  • Hydrotherapy has been considered one of the best alternative therapies to encourage spinal / Truncal control and balance.
  • Ayurveda
  • Unani
  • Acupuncture
  • Acupressure
  • Hippo-therapy
  • Homeopathy

Prognosis

These children should be diagnosed early and treated as babies on neurological lines. The result of early treatment can be surprising. Children with less severe involvement of hands and trunk should walk with the use of elbow crutches. They may later need to use a wheelchair or motorized vehicle. With supreme effort a large number of children can walk without any support and attend normal school. Others, due to the need for treatment and special chairs and appliances during school life, may need education in a special class.

Employment

Employment in later life is influenced by the presence of associate factors. If the child has below average mental ability and poor form of mobility then the chances of open employment is very less. A large number of children can work in a shelter workshop under supervision. Only a few of spastic quadriplegic children can have the opportunity to work in open market, in a factory or a place where mobility is must.

 

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