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Cerebral Palsy

1 ) DEFINATION

CP is a poorly and slowly myelinating syndrome. Non-progressive central motor deficeit due to prenatal or perinatal causes resulting in hampered myelination.

2 ) PATHOGENESIS

CP is an Ischaemia Reperfusion Injury (IRI), i.e. stoppage of blood supply to the organ and restarting blood supply after the gap of some time (may be in minutes to hours depending on type of tissue and disease). In CP, it may be of 5 to 20 minutes, during which the brain, thymus and thyroid are affected the most, as they are high redox organs (require high O2 Supply). The damage is more to white matter (Myelination) of Brain.

In IRI, paradoxically, the organ is damaged not during lack of blood supply, but rather damaged heavily on restarting of blood supply due to heavy production of oxygen radicals (super oxide, hydroxyl radical and H2O2) due to overactive enzyme xanthine oxidase. These radicals damage the tissue and more than that, they damage brain stem cells / NSC / oligodendrite progenitor cells (OPG), which have capacity to repair and regenerate brain. So, myelination process is slowed down (due to damaged OPG cells). This is the basic pathology of CP. This delays maturation of CNS, especially those functions that depend heavily on myelination e.g. motor functions and speech. Myelination is the covering of the neurons (Wires of Brain) providing insulation to CNS electrical activity and thereby increasing speed of brain up by 10 to 100 times more.

Due to poor myelination, fast electrical neural activity like muscle movements and speech coordinations are delayed & do not develop fully. So poor and slow in coordinated motor muscle movements and poor speech development is there. Also, squint in eyes and bizarre movements are there. Slow Brain motor function with normal spinal motor functions is the cause for spasticity and athetosis & hypotonia.

Damage to thymus is the damage to immune system through poor development of T-Iymphocytes.

Thyroid has an important function of maturing CNS through myelination. So damaged thyroid delays myelination further, thereby aggravating mental retardation, defects in movements and speech.

We need to correct all above to cure or palliate CP. Difference between CP and non CP child is in level of myelination (insulation) of nerve fibers of all parts of Brain. The brain is formed during early embryonic phase; and growth and maturation occurs mainly around perinatal period. The myelination is insulation of nerve fibers by wrapping up layers of oligodendrocytes. Myelinated and non-myelinated fibers differ mainly in speed of conduction of electrical messages along the nerve cells by the factor of 10 to 100.
Un-myelinated fibers have conduction speed of 1 to 5 meters per second, whereas myelinated fibers have speed of 100 meters per second.

The new-born child cannot sit, walk or speak immegiately after birth, as his un-myelinated motor nerves are too slow for coordinating different muscles for sitting, walking and speaking! (The cow has calf running fast even after birth!). After birth, myelination occurs fast up to 2 to 5 years of age and continues till adulthood.

3 ) Aetiology

Incidence 2 to 3 per 1000 births
Anything hindering myelination causes or aggravates CP.
a ) Obstetrical complications, affecting oxygenation of the foetus around perinatal period.

  • IUGR
  • Antenatal bleeding
  • Twins
  • Prematurity
  • Prolonged / obstructed labour
  • Prenatal, perinatal & postnatal injuries due to vascular insufficiency, toxins and infections.
  • Birth asphyxia
  • Hypothyroidism /  I2  deficiency in the mother

(Thyroxine is essential for proper myelination)
b ) Infection of Brain if postnatal;  Meningitis / encephalitis damage developing myelinating fibers heavily
c ) Kernicterus – High levels of bilirubin concentrate in basal ganglia (regulates muscle tone and movements) as bilirubin is lipophilic and white matter (mainly made up of fibers 2 to 3 layers of myelination sheath) has high fat content. So bilirubin dissolves in fat in myelin sheath and damages it.

 

 

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Dr. M. B. Sharma Dr Mb Sharma, MBBS, MD (Gynaecology) and he is one of the most popular Tele-Medicine consulting Doctor..
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