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Progressive Neurological Deterioration
Several severe but fortunately rare nervous system disorders first appear in infancy and early childhood. In these disorders an ongoing problem permanently damages nerve cells and worsens over time. In many cases, early death is the outcome; in others, the child lives longer, but with progressive disability.

The nervous system, which controls our senses and coordinates all of our activities, body functions and thinking is divided into two parts. The brain and spinal cord make up the central nervous system, which issues directions for all body activities and processes information gathered from the body and the outer world. The peripheral nervous system, which is the network of nerves branching out to the muscles, sense organs, and internal organs, provides communication lines necessary for proper functioning of the five senses, voluntary motor activities (such as walking and throwing a ball) and involuntary processes (such as heartbeat, breathing and digestion).

WHAT CAUSES PROGRESSIVE NEUROLOGIC DETERIORATION?
Inherited deficits in enzymes or other essential body substances are often at the root of the problem. Some of these metabolic abnormalities affect body systems on so many levels that they are called protean diseases. Other cases of progressive neurologic deterioration are brought about by infection, and the cause of still others remain to be discovered.

WHEN SHOULD I SUSPECT THAT LOSS OF SKILL OR SLOWING OF MATURATION IN ANY AREA OF DEVELOPMENT MAY SUGGEST A PROGRESSIVE NEUROLOGICAL DISORDER?
The child may develop a severe seizure disorder or suffer from uncontrollable small jerking movements. Weakness, poor coordination and movement disorders may occur. Changes in personality or loss of vision or hearing may occur as well.

The symptoms are complex and depend on the cause as well as on which nerve cells (neurons) are involved. When cells that control voluntary motion (called the upper motor neurons) are damaged, muscles may become spastic, movements stiff and reflexes abnormal. Diminished muscle tone and selective muscle wasting may accompany deterioration of lower motor neurons, which transmit signals to and from the peripheral nervous system. For descriptions of some of these symptoms, see the chart on "Disorders causing progressive neurological deterioration."

HOW IS PROGRESSIVE NEUROLOGIC DETERIORATION TREATED?
Treatment depends on the cause. In general, however, cures are rarely possible. In most cases, the focus is on providing physical, occupational and behavioral therapies, orthopedic devices and medications to control seizures.

DISORDERS CAUSING PROGRESSIVE NEUROLOGIC DETERIORATION
Duchenne Muscular Distrophy

• Symptoms:
 Weakness beginning in the legs and progressing through shoulders and trunk; delayed walking.

• Age of onset:
3 to 5 years

Infantile Spinal Muscular Distrophy Type 1 (Werdig-Hoffman Disease)

• Symptoms:
Inactivity; weak shoulders; feeble cry; knees flexed; does not learn to roll over or sit.

• Age of onset:
Birth to two months (genetic disease)

Infantile Spinal Muscular Distrophy Type 2

• Symptoms:
Weakness first in arms and legs, later more generalized; breastbone may be depressed; little movement during sleep or relaxation; may learn to stand if holding something.

• Age of onset:
2 to 12 months (genetic disease)

Infantile Spinal Muscular Distrophy Type 3

• Symptoms:
May start normally, learn to sit unassisted from 6-8 months; develop weak thighs and hips but may learn to walk; waddling gait; outward curve of lower spine, protruding abdomen; may need wheelchair by age ten or later.

• Age of onset:
During second year (genetic disease)

Juvenille Spinal Muscular Atrophy (Kugelburg-Welander Disease)

• Symptoms:
Slowly progressing weakness starting in shoulders, thighs and hips; may develop abnormal gait and curve of lower spine; may continue to walk or use a wheelchair later in life. May live normal lifespan.

• Age of onset:
2 to 17 years (genetic disease)

Rett Syndrome

• Symptoms:
First appear normal, then rapid decline of useful hand movement and speech. Hands begin making repeated gestures such as wringing and tapping. Little eye contact; abnormal breathing; seizures; failure to grow; progressive immobility.

• Age of onset:
Girls only, 6 to 18 months (maybe genetic)

Adrenoleukodystrophy

• Symptoms:
Personality changes; problems with schoolwork; problems in movement; sometimes seizures; progressive slowing of physical and mental reactions; stiff, awkward movements; vision loss.

• Age of onset:
Boys only, 3 to 16 years

Excerpt from THE DISNEY ENCYCLOPEDIA OF BABY & CHILD CARE, © 1995 DSH Communications, Inc. This material is based on current medical research and, to the best of the editors' knowledge and understanding, is accurate and valid. However the reader should not use information contained in this material to alter a medically prescribed regimen or as a form of self-treatment, without seeking the advice of a licensed physician.

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