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.
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.
This is the actual group of children identified by Dr Little. The management / treatment of this group are very rewarding. Early Intervention program should be started as soon as the child is diagnosed clinically. These children can have very good prognosis if they are treated with combination therapy / Integrated Approach.
a. Handling, Transfer and Carrying Technique
Mostly these children prefer to sit in ‘W Sitting” posture. This pattern should be avoided as much as possible. While carrying the child, it should be tried to keep the hips abducted if possible.
b. Use of Aids and Appliances
The child should be encouraged to sit arm chair with pommel so that the child does not start w sitting. Generally these children do not require the support of standing frame as their trunks are mostly stable.
The role of Orthotic aids is very high in this group of children. Preventive AFO should be used while night as early as possible. The corset gaiters should be replaced with 3-points gaiters as the child becomes 3 years old. If the child shows sign of tibial torsion, the gaiters should be modified to work as anti-torsion splint. A few children with hyper-extended knees may be helped with modified AFO. Always remember, there is no place for HKAFO / Calipers / KAFO in the current management technique so these should be discouraged as far as possible). In few cases GRFO can be tried although there is little data available to support the use of GRFO.
Mostly children of this group require Posterior Rollator rather Anterior Rollator. Researches have demonstrated the efficacy of the posterior rollator higher than anterior rollator. Parents / Professionals should follow the FMS (Functional Mobility Scale) guidelines as much as possible.
Physiotherapy plays a great role in the whole management. The components of the program should have Breathing exercises, Alignment therapy, strength training and balance exercises.
d. Developmental Therapy
Postural development should be started with prone development. The program should consist of postural enhancement techniques through static posturing, dynamic posturing, and Transition. To get good dissociation and stabilization, trained therapist can include the Neuro-enhancing postures also. Mobility training should be the crux of the program. Starting from pivoting on prone to independent walking with optimal energy expenditure should be ultimate goal of the whole developmental therapy.
e. Occupational Therapy
Mostly Children with Spastic Diplegia have very mild problems with upper extremity but in the long run, they face problems with fine motor skills such as buttoning / unbuttoning and writing. Generally, parents of these children do not feel any problems with hand function in the early fays so they do not ask for hand function training. Professionals should explain the parents about the importance of hand function in the total management.
f. Special Education
In general children with spastic diplegia are mentally normal and intelligence but a few children can have associated problems like Mental Retardation, Epilepsy, Visual impairment or Perceptual Problems. These children will be benefited with special education.
g. Speech Therapy
A few children with CP Spastic Diplegia can have speech impairment, so they should be provided speech therapy. The speech therapist should work on drooling and feeding disorders also if it is required.
h. Role of Oral Medicines
A few children with associated disorders may require oral medications. Anti-spastic medicines are generally prescribed for this group of children but there is a controversy about the need of theses anti-spastic medicines. Researchers feel if the child has focal spasticity so there in no need to go for oral anti-spastic drugs.
i. Role of Chemodenervation
The role of Chemodenervation has a definite place in the total management of this group of children. Generally, these children require Botox (Botulinum toxins) for relaxing Hip Adductor Tightness, Knee Flexors Tightness, Plantar flexors Tightness. Very rarely, these children require Botox for Hip flexors or Midarm Supinators Tightness. Post Botox therapy is the key of success in case Botox Injected children.
j. Orthopedic Surgery
Orthopedic surgery has a definite role in this group of children. Older Children who have true equinus, Knee flexion contracture, Hip Adductor contracture etc require soft tissue release. In case of bony deformity, these children undergo bony procedure (osteotomy). The result of the procedure depends on the quality of post operative therapy. Mostly surgeries fail due to poor therapy protocol. Parents should identify the post operative therapist before they find an orthopedic surgeon for the procedure.
Rhizotomy plays great role in selected cases of CP Spastic Diplegia. A good assessment and procedure can do miracle but a bad decision can make the child cripple also
l. Intrathecal Baclofen
Very few cases of this group of children require the help of ITBP. Spasticity can easily managed with other available procedures.
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 Diplegic 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!
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.
- Hippo-therapy- Hippo-therapy has shown good response with this group.
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.
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.