BABY AND CHILDHOOD NERVOUS SYSTEM AND MUSCLE DISORDERS: DEGENERATIVE DISORDERS OF THE NERVOUS SYSTEM
Various diseases of the nervous system may take place which are caused by the outer insulating material of the nerve fibres becoming destroyed. Often these are not diagnosed at birth, for the baby appears normal when born. They may start insidiously as the child becomes older, and the symptoms gradually worsen. There is frequently a family history of the same disorder.
Fortunately, they are not common, and many doctors will see few if any during their professional lifetime. They tend to wind up at the larger hospitals where, because of their vagueness, they have been referred for investigation.
Brief mention only will be made of some of them.
Demyelinating encephalopathy
Demyelinating encephalopathy (or Schilder's disease) means the insulating material in the brain deteriorates. This leads to paralysis, firstly the limbs, then in the trunk. There are headaches, vomiting, deafness, convulsions and mental deterioration. No treatment is available.
Tay Sachs disease
This occurs in young babies. It is due to granules of fat being deposited in the nervous system between the third and sixth month of life. Apathy, lethargy and weakness take place. The child usually dies by the age of 3 years.
Hand-Schuller-Christian syndrome
This comes on insidiously, with lethargy, a discharge from the ears, loss of teeth and swellings over the skull or other bones. Internal organs may be adversely affected. It may smoulder on for many years, and may be cured spontaneously or by the use of steroids.
Gaucher's disease
Gaucher's disease is a rare disorder inherited from parents, more common among Jews. It may come on at any age. An enlarged spleen, skin discoloration and haemorrhages from the nose and pain in the limbs may take place. There is little satisfactory treatment, and although it may smoulder on for years, most infants die.
Friedreich's Ataxia
This also is an inherited nervous system disease which may come on between the age of 5 and 15 years. The legs are firstly affected with jerky walking movements. The speech becomes jerky and indistinct, and the arms become affected. Most children die within 10 to 15 years.
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