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Cerebral Palsy Treatment in India

What is Cerebral Palsy

Cerebral Palsy (CP) is a Neurodevelopmental Disorder affecting about 1 to 4 children per 1000 new born, characterized by abnormal low or high muscle tone along with inadequate control over muscles, associated with variable disability in Cognitive abilities, hearing and sight, and sometimes autism like behavior. There has been no change with modern medical advances in reducing the incidence of CP but with Cerebral Palsy Treatment in India has seen advances. Earlier, CP was thought to be mainly due to poor obstetric care and management, but epidemiological studies have refuted this.

Main Causes

Brain damage before, during or within 2 years of child birth causes CP

Maternal infection, diabetes, high blood pressure, Hypothyroidism

Birth trauma or asphyxia

Post-partum brain trauma or severe infection.

Disturbance or inadequacy of Umbilical Blood Supply

Other Causes

Multiple gestation,

Prematurity,

Low birth weight,

Genetic (?) thrombophilic disorders

Fetal inflammatory response to viral or bacterial infections during pregnancy

Intrauterine abnormal fetal cytokine responses, etc.

  • Gibson, Catherine S et al, Obstetrical & Gynecological Survey:2003; 58(3): 209-220

 

 Is Genetics involved?

There is increasing evidence that Genetics is not entirely to be ruled out in cases of CP, as some reports show.

Child homozygous for the thrombophilic factor-V Leiden (fVL) mutation develop hereditary clotting tendency in many organs including brain. This may cause CP.

  • KH Harum et al, Developmental Medicine & Child Neurology, 1999, 41(11): 777-780

Argininemia is a treatable genetic cause of progressive spastic diplegia simulating cerebral palsy

  • AN Prasad et al, J Child Neurol August 1997 vol. 12no. 5 301-309

High quality Brain MRI may show important diagnostic clues in distinguishing genetic-metabolic disorders from other causes. Hypoxic-ischemic encephalopathy at the end of a term gestation, produces a characteristic pattern of hyper intense signal and atrophy in the putamen and thalamus.

 

Signal abnormalities & atrophy of brain parts in the putamen, globus pallidus, or caudate can point to genetic-metabolic diseases, including disorders of mitochondrial and organic acid metabolism.

  • AH Hoon et al, J Pediatr 1997;131:240-5)

 

Comprehensive Assessment for CP

Assessment should be comprehensive considering the total child and his normal environment. It includes the following, in a child-specific need

Gross motor function

Fine motor and hand function

Postural reactions

Handwriting assessments

Cognitive and perceptual assessment

Play assessment

Social skills assessment

It is not out of place to remind that CP is not just a Motor Associated Problem. It is associated with:

Speech Impairment –    in about 25%

Visual Impairment –       in about 25%

Mental Retardation –     in > 25%

Sensory Dysfunction –   in about 2%

Neurological association with function

Part of Brain  Function regulated Age
1: medulla moving arms and legs birth
2: pons cross-pattern crawling 3m
3: midbrain creeping 6m
4: cortex walking with arms in a primary balance 12m
5: cortex walking free from primary balance 18m
6: cortex: crosspattern running 36m
7: cortex: using a leg in a skilled role, consistent with the dominant hemisphere 72m

Scales for Examination and Assessment of Disability

There are many types of measurement scales used to assess the ability of the child on various parameters, not all perfect, especially those for Special Education and Speech, since:

The child may understand but be unable to perform

The child may be able to perform only if he/she could understand

A variable degree of dysfunction and inadequacy of both.

However, the following scales are often used by specialty centers

Muscles tone (on Modified Ashworth’s scale)

Deep tendon reflexes / Primitive reflexes

Ankle / Patellar clonus

Tightness / Contracture/ Deformities

Voluntary motor control

Balance / Equilibrium

Hand functions

  • Pre –writing skills

GMFCS
(Gross Motor Functional Classification System)

  • This is a classification System for classifying disability as per age into five classes, from 5 as severest form to 1 as mildest form.
  • Therapy induced change by even one level to higher level (= lesser number) is significant

GMFM
(Gross Motor Functional measurement)

GMFM done on 88 parameters shows motor abilities in five domains in lower limbs:

  1. Lying & Rolling
  2. Sitting
  3. Crawling & Kneeling
  4. Standing
  5. Walking ,Running & Jumping

GMFM done on 66 specific parameters gives an overall score of serial changes in lower limb action.

QUEST
(Quality of Upper Extremity Skills test)

  • Givew results similar to GMFM, but for upper extremity.
    1. Shoulder items
    2. Elbow items
    3. Wrist items
    4. Finger Movements
    5. Grasps

Other common tests

  • REEL for Speech, Language & Communication ability, and BASIC MR, FAB, ADOS, RUTH-GRIFFITH, etc. give an idea about Special Educational parameters. However, they are not mathematical like GMFM & QUEST.

Management

HOME BASED THERAPY PROGRAM

Positioning

Weight Bearing (if needed)

Active ROM exercises of all joints 5 times each joint

ADL & Hand Function to eat properly, hold and release properly, dress and undress properly, signal for or independently perform toilet needs

Standard Rehabilitation Therapy

Physiotherapy for strengthening the body and mobilizing bones and joints

Occupational Therapy to teach the body proper way to function and work

Speech, including Oro-motor Therapy to exercise all structures of breathing, eating and phonation. It includes sucking, chewing, swallowing, saliva drooling control, soft and hard palate control, solid and liquid ingestion, and THEN speech development

Special Education to increase the ability of the child become self-reliant, and a useful member of family, society and country, ability dependent education, coaching Activities of Daily Living (ADL), Potty training, dressing and undressing hand function, eating, etc.

Psychological Counselling and guidance to both the child and parents.

  • Make the parents realize that they have a very important role to play TOGETHER to give their child the best fighting chance in life.
  • That Rehab must continue till bone growth stops (M-21, F-18)

 

Established or Off-label Experimental Early Medical Intervention Therapies

Botox injections give 4 to 6 months of relief from spasticity, and then may need to be repeated or supplanted by Tendon Lengthening Surgery.

  • No cognitive or communication improvement

Acupuncture/Acupressure

  • Gives some improvement in motor control. It is cost-effective when done by a TRAINED therapist. However, it also only provides muscle relief for the duration of therapy.

Selective Dorsal Rhiozotomy Surgery is a skilled brain operation to permanently cut some of the fibers that are causing spasticity, to reduce the symptom on a long term basis. However, here also, no other brain function is improved.

Corrective Surgery is done to lengthen contracted muscles when all other means have failed to straighten the limb, in order to protect damage to the articular surface of bones at the affected joint. Combined with proper Physical Therapies, it is a highly successful surgery to restore gait and movement in intractable contracture. This is different from Polio deformity correction surgery where total correction is done. In CP, doing that would lead to unstable floppy joint function. Correct degree of release is a matter of careful judgment based on experience.

Bramhi, an ancient Indian Ayurvedic / Unani plant extract formulation restores brain dysfunction and damage to an extent, including cognitive ability. However, the preparation needs a very careful manufacturing protocol to ensure a purified standardized extract with correct ratios of specific adjuvant plant extracts as per ancient literature, to achieve the desired potency and efficacy.

Hyperbaric Oxygen Therapy (HBOT) uses Henry’s Law of Physics that stipulates that the solubility of gas in water increases with pressure. The patient with hypoxic or severely inflamed tissues anywhere in the body, including brain is put inside a pressure chamber which is inflated to achieve a pressure of 11 to 16 feet of water (1.3 ATA to 1.5 ATA) and made to breathe ambient air or variable concentration of oxygen as per need. The damaged but not dead tissues get the lifesaving oxygen to revive back into function. The benefits of HBOT can be summarized as follows:

  • Enhances microcirculation and increases new blood vessel formation
  • Marx, RE. Am J Surg, 11/90; 160: 519-24
  • Manson, PN. Surg Forum, 1980; 31: 564-66
  • Improves ischemic tissue oxygen capacitance
    • Siddiqui, A. Plast Reconstr Surg, 1997; 99: 148-55
  • Reduces inflammation and edema
  • Mobilizes Bone Marrow Stem Cells
    • Thom, SR. Am J Physiol Heart Circ Physiol, 2006;290:1378-86.
  • Speeds rate of wound/damage repair
  • Stimulates hibernating / dormant resident stem cells into activity to initiate auto-repair
  • Enhances Neuro-Plasticity and normalizes of metabolic apoptosis & DNA signaling
    • P Harch, Neural Repair & Neuro Rehabilitation Conf. (NR2CON), Ind. Inst. of Technology; Delhi; Sept. 2007
  • Increased Fibroblast Proliferation
    • Hehenberger, K. Wound Rep Regen, 1997; 5: 147-50
  • Increases Bone Mineral Density & Healing
    • Ueng, SWN. J of Trauma; Injury , Infection & Critical Care, 1998; 44(4): 676-81
  • Increases Epithelialization
    • Uhl, E. Plast Reconstr Surg, 1994; 93: 835
    • Manson, PN. Surg Forum, 1980; 31: 564-66

Stem cell Therapy

  • Stem cells are the remnant colony of the original germ cells that gave rise to the person. Hence they are called Master Cells of the body. Undifferentiated cells that have the ability to divide & differentiate into many other cell types. They are distinguished from other cell types by two important characters
  1. Capable of renewing themselves through cell division, sometimes after long periods of inactivity.
  2. Can be induced to become tissue or organ specific cells with special functions, like cartilage, liver tissue, heart muscle, pancreatic tissue, nerve tissue, blood cells etc.
    • Embryonic Stem Cells are obtained by killing a fetus and obtaining stem cells from its various tissues. It is associated with many legal, religious, moral and ethical issues, hence generally looked down upon. They can make a very wide variety of tissues.
    • Stem cells obtained from an adult or a child after birth, hence more acceptable. They are called “Adult stem cells”. They have limited functionality, meaning specific stem cell types can only mature into a selected number of tissue types.
    • Mechanism of Action: Different source and different types of stem cells exhibit the following benefits:
      • Glial derived neurotrophic factor (GDNF) release can rescue neurons from a lack of oxygen and help repair of brain white matter
      • Specific types of stem cells enhance the release of Neurotrophin 3 (NT-3) Nerve Growth Factor (NGF) and Brain Derived Neurotrophic Factor (BDNF) to stimulate growth of new neurons.
      • Stem cells support existing injured neurons by attaching to them. This type of “cell fusion” may be more efficient than creating new neurons in cases where neurons may have hundreds of synapses already established
      • Stem cells stimulate VEGF (Vascular Endothelial Growth Factor) release that promotes growth of new neurons to repair and replace injured brain tissue.
      • Some types of stem cells have been found to give rise to a small percentage of neurons that can repair the gray matter of the brain and spinal cord.
    • Help balance the immune system and autoimmune disorders.
    • Relax the artery walls which promotes improved blood flow.
    • Safety of adult stem cells has not been questioned seriously so far in spite of many decades of use (in marrow replacement therapy for blood cancer). However, the use of embryonic stem cells has been noted to cause tumor growth after 4 to 6 years in a very small number of patients.
    • Intensive Standard Therapies THROUGHOUT is mandatory for optimum benefit.
    • Follow up to show efficacy requires 2 monthly checkup for at least 2 years, with the first few months under close supervision of the medical institution.

Repetitive Transcranial Magnetic Stimulation Therapy (rTMS).

    • rTMS uses an electromagnetic coil to give stimulation in a pulsed manner to specific affected parts of brain.
    • The intensity and duration of stimulation, number of pulses per sitting, number of sittings and duration of time in-between one round, is still under investigation.
    • rTMS reduces muscle spasticity and may have a slight beneficial effect on Cognitive skills as well.