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CEREBRAL PALSY RIGIDITY



General Features
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.

Common Causes
Abnormalities in the birth process are much less in evidence. In some children the condition is due to a recessive gene, and this may be realized only after the birth of a second affected child, although the first child was born after an abnormal birth - a factor that must have been irrelevant to the condition. Some children are light for dates, suggesting defective development in utters. There is often evidence of other congenital abnormalities.

Associated Factors
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.

Early Identification If the child has some form of congenital abnormalities than it is easy to identify the condition otherwise these children are diagnosed after 4 months when they cannot reach to a desired object and there is a strong presence of STNR.

Treatment

TREATMENT

The management of children with CP Rigidity is very much similar to the management of children with CP spasticity. But there is a great difference in the physiotherapy protocol. Hippo-therapy and hydrotherapy have a special place in the management program. These children are considered more disable than the children with spasticity. Due to their good intelligence, treating these children is very rewarding. 

Standard Techniques
a. Handling, Transfer and Carrying Technique
These children should be handled scientifically to get maximum dissociation and stability in the postures.

b. Use of Aids and Appliances
Postural Aids
These children require help of these postural aids according to the milestones achieved. Corner chair with tray cut out, Arm chair with tray cut out, Standing frame, etc.
Always remember to place a pommel in the chair / standing frame to prevent hip subluxation / dislocation.
Orthotic Aids
AFO, SMO, Knee immobilizer, Cock-up splint, arm band, etc are generally used need based.
Mobility Aids
 Prone scooter, crawler, Rollator, Elbow crutches, Sticks, etc can be used in case of need of aids oriented mobility.

c. Physiotherapy
The components of the PT program should have Alignment Program, Stretching Program, and Strength Training Program

d. Developmental Therapy
The child should be encouraged to achieve milestones progressively from head control to standing. Dissociation to get transition is the key of success in developing milestones.

e. Occupational Therapy
These children have very poor dissociation and stabilization so the program should be designed in a way where the children get confidence to use their potentials to be independent in life.

f. Special Education
Special education has a definite role in the total development of the child. Although these children have mostly good mental level but they do not performance according their mental level due to disobedient body.

g. Speech Therapy
Due to rigidity, these children have problems with speech quality. A speech therapist can play a vital role here to improve communication.

h. Role of Oral Medicines
Generally medicines are required for Epilepsy, and drooling. Anti-spastic drugs like Baclofen, Tizanidine etc are also prescribed to manage the rigidity.

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 Botox therapy is not available in most of the physiotherapy centre that is why the role of Botox has been underestimated in the management of spasticity / rigidity in cerebral palsy.  
  
j. Orthopedic Surgery
Due to bad postures, these children get bony deformity very early. So an orthopedic surgeon should be included in the management team as early as possible. Hip surveillance program should be integrated.  

k. Rhizotomy
Selective dorsal Rhizotomy is one of the available treatments which can be used in a few selected cases of CP Rigidity.  

l. Intrathecal Baclofen
Intra-thecal Baclofen therapy is another option of treatments for these children.

Experimental Therapies Experimental Therapies
 A. Hyperbaric Oxygen Therapy (HBOT)
As HBOT is a generalized treatment for children with cerebral palsy, Children with Rigidity 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.

B. Stem Cell Therapy (SCT)
Stem cells are considered repairing cell of the body. As the children with CP Rigidity 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. Homeopathy
  2. Ayurveda
  3. Unani
  4. Acupuncture
  5. Acupressure
  6. Hippo-therapy- Hippo-therapy has shown very good effect on dissociation and stabilization in these children
  7. Hydrotherapy –Hydrotherapy has been considered a good option to get easy dissociation.   


Prognosis

Employment
Although these children are good in academics but due to poor hand function they are not considered good candidate for open market employment. But with good computer training, they can be placed in offices or shops for computing.

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